Acute megakaryoblastic leukemia, or type 7 (AML 7), is a rare entity defined by a proliferation of blasts of which at least 50% are megakaryoblasts. It represents 0.5 to 2% of acute leukemias (LA) in children and 3 to 10% of acute myeloid leukemias (AML). Patients may have nonspecific symptoms. His diagnosis is based on the presence in the blood or in the marrow of blasts with typical morphology and expressing specific platelet antigens. This observation describes the clinical case of a 19-month-old infant with pancytopenia. The diagnosis of AML7 was made based on explorations of peripheral blood, myelogram, and immunophenotypic analysis. Chemotherapy has been started, with a poor therapeutic response. The patient died in a state of septic shock. We recall through a literature review the clinical, histological and immunophenotypic features of acute megakaryoblastic leukemia.