Philadelphia chromosome-like acute lymphoblastic leukemia (BCR-ABL1-like ALL) is a newly defined ALL subtype in 2009. It is Philadelphia chromosome (Ph)/BCR-ABL1-negative and characterized by a set of gene expression profile which is highly similar to that of Ph/BCR-ABL1-positive ALL. However, there is no definitive unified diagnostic criteria yet. BCR-ABL1-like ALL is generally resistant to chemotherapy, with a high relapsed rate and poor prognosis. It harbors a diverse range of genetic alterations that affect cytokine receptor and/or signal transduction pathway of tyrosine kinase. Overexpression of CRLF2, JAK-STAT pathway abnormalities and ABL-class gene rearrangements are the most common. These genetic aberrations could be therapeutic targets. Both in vitro and in vivo experiments and clinical data support the efficacy of targeted therapy. Beside conventional multi-drug chemotherapy, the combination of targeted therapy, cellular immunotherapy and allogeneic hematopoietic stem cell transplantation is promising to improve the prognosis of BCR-ABL1-like ALL. In this paper, the research status of BCR-ABL1-like ALL is described from five aspects: definition, diagnosis, clinical characteristics, molecular biological characteristics and treatment.