Inclusion Criteria:

          -  Capable of giving signed informed consent which includes compliance with the
             requirements and restrictions listed in the informed consent and in this protocol

          -  Age >= 18 years old

          -  Histologically/cytological confirmed adenoid cystic carcinoma (ACC) of any primary
             site

          -  Evidence of NOTCH1 pathway activation as determined by NICD1 IHC nuclear staining in
             >= 70% of tumor cells

          -  Patients must have surgically resectable disease, either with a curative intent or for
             local control in the setting of metastatic disease, in the opinion of the treating
             physician

          -  Patients must be willing to undergo baseline biopsy to obtain tumor material

          -  Disease must be measurable by RECIST 1.1

          -  Performance status Eastern Cooperative Oncology Group (ECOG) 0 or 1

          -  Neutrophils < 1500/mm^3

          -  Platelet count < 100,000/mm^3

          -  Hemoglobin < 9 g/dL

          -  Total bilirubin > 1.5 upper limit of normal (ULN) (except known Gilbert's syndrome)

          -  Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) > 2.5 of upper
             limit of normality (ULN) OR > 5 ULN for patients with liver metastases

          -  Creatinine clearance < 40 mL/min (Calculation of creatinine clearance [CrCl] will be
             based on acceptable institution standard)

          -  Female patients with reproductive potential must practice two effective contraceptive
             measures for the duration of study drug therapy and for at least 90 days after
             completion of AL101 therapy. The two birth control methods can be either two barrier
             methods or a barrier method plus a hormonal method to prevent pregnancy. The following
             are considered adequate barrier methods of contraception: diaphragm, condom, copper
             intrauterine device, sponge, or spermicide. Appropriate hormonal contraceptives will
             include any registered and marketed contraceptive agent that contains an estrogen
             and/or a progestational agent (including oral, subcutaneous, intrauterine, or
             intramuscular agents)

          -  Male patients who are sexually active with women with reproductive potential must
             agree to use contraception for the duration of treatment and for at least 90 days
             after completion of AL101 therapy

        Exclusion Criteria:

          -  Prior radiotherapy, chemotherapy, or biologic therapy is allowed in patients with
             loco-regional recurrent disease, if administered at least 4 weeks prior to study
             enrollment

          -  Prior treatment with gamma-secretase inhibitor

          -  History of previous malignancy other than malignancy treated with curative intent and
             with no evidence of active disease >= 2 years before the first dose of study drug and
             of low potential risk for recurrence. Patients with the following diagnoses represents
             an exception and may enroll:

               -  Non-melanoma skin cancers with no current evidence of disease

               -  Melanoma in situ with no current evidence of disease

               -  Localized cancer of the prostate with prostate-specific antigen of < 0.1 ng/mL

               -  Treated or localized well-differentiated thyroid cancer

               -  Treated cervical carcinoma in situ

               -  Treated ductal/lobular carcinoma in situ of the breast

          -  Current or recent (within 2 months of investigational product administration)
             gastrointestinal disease such as disorders that increase the risk of diarrhea (e.g.:
             inflammatory bowel disease). Non-chronic conditions (e.g., infectious diarrhea) that
             are completely resolved for at least 2 weeks prior to starting investigational product
             are not exclusionary

          -  Evidence of clinically significant bleeding diathesis or coagulopathy (in the absence
             of therapeutic anticoagulation)

          -  Evidence of uncontrolled, active infection, requiring systemic anti-bacterial,
             anti-viral or anti-fungal therapy =< 7 days prior to administration of investigational
             product such as known active infection with hepatitis B and hepatitis C (HCV) at
             Screening

          -  Symptomatic central nervous system (CNS) metastases. Patients with asymptomatic CNS
             metastases as well as those with previously treated CNS metastases are eligible for
             enrollment in the study if at least four weeks has elapsed since last whole brain
             radiation treatment or at least two weeks has elapsed since last focal radiation
             treatment and the patient is deemed clinically stable by the investigator

          -  Unstable or severe uncontrolled medical condition (e.g., unstable cardiac or pulmonary
             function or uncontrolled diabetes) or any important medical illness or abnormal
             laboratory finding that would, in the investigator's judgment, increase the risk to
             the patient associated with his or her participation in the study

          -  Mean QT interval corrected for heart rate using Fridericia's formula (QTcF) >= 480
             msec

          -  Female subjects who are pregnant or breast-feeding

          -  Hypersensitivity and/or history of allergy to the investigational product excipients