A case of di Guglielmo syndrome in 9 year old boy was presented. This syndrome is characterized by excessive erythroid precursors with abnormal morphology both in the bone marrow and blood, and rare in childhood. He visited with the chief complaints of general weakness, weight loss, epistaxis and abdominal distension. Physical examination showed pallor, grade I-II/VI systolic murmur on apical area and marked abdominal distension with hepatosplenomegaly. we confirmed the patient by the peripheral smear and bone marrow aspiration.