Background: Acute leukemia area a group of neoplastic disorders characterized by proliferation and accumulation of immature hematopoeitic cells in bone marrow, blood, and other tissues. The present study was conducted to have a detailed understanding of immunophenotyping profi le, the morphologic and immunophenotypic discrepancy and importance of immunophenotyping in diagnosis of acute leukemia. Objectives: To study immunophenotyping profi le in acute leukemia (acute myeloid leukemia [AML], acute lymphoid leukemia, and mixed lineage leukemia) and to study its importance in diagnosis. Materials and Methods: This study was performed in Medical College, Jabalpur. 160 patients diagnosed morphologically with AML, acute lymphoblastic leukemia and mixed lineage leukemia seen were included in the study. Results: Only in 73% cases of acute leukemia did fi nd similarity in morphological appearance and immunophenotyping. In remaining 27% cases morphological fi ndings did not correlate with immunophenotyping expression. Diagnosis in these 27% patients changed after immunophenotyping. Conclusions: It is imperative and absolutely essential to ascertain the lineage of leukemia by immunophenotyping before starting on treatment as more than 25% of patients would not respond or later relapse if treatment is initiated on morphological diagnosis.