We report the case of a 2-year-3-month-old boy with acute megakaryoblastic leukemia showing hemophagocytosis by leukemic blasts. The chromosome analysis of his bone marrow revealed t(16;21)(p11;q22). In addition to the present case, we found 4 other acute myeloid leukemia (AML) cases associated with hemophagocytosis and t(16;21)(p11;q22) in the literature, of which 3 were megakaryoblastic. Although the syndrome of AML with FAB-M4/5 morphology, t(8;16)(p11;p13), and erythrophagocytosis is well known, leukemic blasts of FAB-M7 morphology showing t(16;21)(p11;q22) may be underscored for their phagocytic activity.