Philadelphia chromosome positive (Ph+) in T-lineage acute lymphoproliferative tumors is a rare event in both children and adults. In particular, it has not been reported in T-cell lymphoblastic lymphoma(T-LBL) yet. Here, we describe a patient with Ph+ T-LBL for both cytogenetic abnormality and BCR-ABL1 fusion transcript. Moreover, we review the published cases of Ph+ T-cell acute lymphoblastic leukemia (T-ALL) in the literature and summarize their clinical characteristics, management, and prognosis.