We report a case of acute myeloid leukemia with morphologic features of M7 according to the FAB (French-American-British) classification and severe eosinophilia in the peripheral blood and bone marrow at diagnosis. We consider it as congenital leukemia, as the symptoms started in the first month of life of the affected child. This case of leukemia is characterized by t(3;4;6)(q26;q25;q21) cytogenetic abnormality. The blasts in flow cytometry analysis expressed markers of megakaryocytic lineage along with expression of myeloperoxidase in 30% of them. This type of acute myelogenous leukemia with severe eosinophilia can be considered as a distinct clinicopathologic entity.