A case of acute biphenotypic leukemia with mixed blast morphology and combined myeloid and T-lymphoid features is reported. The leukemic cells consisted of small to medium sized hand mirror shaped blasts and large blasts with cytoplasmic granules and some cytoplasmic inclusion bodies. The blast cells were found to be immature T-lymphoid cells(CD2+ and CD7+) that also expressed the myeloid antigens such as CD13 and CD33. In the review of the literatures, additional cases of acute mixed leukemia-hand mirror variant show strong expression of adhesion moleules such as CD2, CD7, and CD11b. Cytogenetic studies revealed a trisomy 4 previously described in acute undifferentiated myeloblastic leukemia with hand-mirror cells. This case represents a morphologically and phenotypically distinct subtype of acute biphenotypic leukemia with hand mirror morphologic features. Adult acute leukemia cases with hand mirror morphology should be considered the possibility of mixed lineage leukemia and processed further studies.