Acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL) are distinct subtypes of acute leukemia with respect to clinical and genetic features. Recently, ALLs were reported to share similar genes like IDH1 mutations to AMLs. Specifically, mutated IDH1 (5%-15%) frequently occurs in AML and scarcely (1.9%) in ALL. Notably, IDH1 along with SRSF2 mutation often exists in secondary AML patients and confers a poor prognosis. Recently, venetoclax (VEN) has been demonstrated extremely effective in combination with a hypomethylating agent or chemotherapy for IDH1 mutated AML patients. However, whether VEN combining chemotherapy is effective among ALL patients with similar genetic features to AML remains unclear. Here, we successfully rescued a relapsed ALL case with IDH1 mutation by VEN plus methotrexate and pegaspargase based on their antimetabolic pathways.