Acute megakaryoblastic leukemia is a very rare type of leukemia characterized by systemic and rapidly progressive proliferation of atypical and primitive megakaryocytes and their undifferentiated precursors. A case of acute megakaryoblastic leukemia in a 11/2 year-old boy was presented. He had marked anemia and thrombocytopenia and normal white blood cell count with a small number of blasts in peripheral blood. Hepatosplenomegaly was noted. Bone marrow revealed massive proliferation of leukemic blasts admixed with a small number of giant cells suggestive of immature megakaryocytes. PAS stain showed characteristic heavy blocks of PAS positive material in the majority of leukemic cells. As far as we can determine, this is the first such case to be reported in the Korean literature.