Acute megakaryoblastic leukemia (AMKL) is a subtype of acute myeloid leukemia (AML) accounting for 3%–10% of primary AML in childhood. Clinical manifestations of AML patients can include low grade of fever, diarrhea, easy bruising, failure to growth, and life-threatening clinical manifestations. Laboratory tests are very crucial to make a definitive diagnosis and treatment. We report here an uncommon case of AMKL in a 12-month-old boy who presented with general paleness and fatigue. Based on blood film investigation, bone marrow examination report, and immunophenotyping, he was diagnosed as a case of AMKL without Down syndrome.