<b><i>Background:</i></b> <i>BCR/ABL1</i>-like acute lymphoblastic leukemia is a newly recognized high-risk subtype of ALL, characterized by the presence of genetic alterations activating kinase and cytokine receptor signaling. This subtype is associated with inferior outcomes, compared to other B-cell precursor ALL. <b><i>Summary:</i></b> The recognition of <i>BCR/ABL1</i>-like ALL is challenging due to the complexity of underlying genetic alterations. Rearrangements of <i>CRLF2</i> are the most frequent alteration in <i>BCR/ABL1</i>-like ALL and can be identified by flow cytometry. The identification of <i>BCR/ABL1</i>-like ALL can be achieved with stepwise algorithms or broad-based testing. The main goal of the diagnostic analysis is to detect the underlying genetic alterations, which are critical for the diagnosis and targeted therapy. <b><i>Key Messages:</i></b> The aim of the manuscript is to review the available data on <i>BCR/ABL1</i>-like ALL characteristics, diagnostic algorithms, and novel, molecularly targeted therapeutic options.