An extramedullary myeloid tumor (EMT) is a gross mass of leukemic tissue at a site other than the bone marrow. The incidence of EMT is reported to be between 20% and 50% at the time of diagnosis of childhood AML [
1,
2]. The highest incidence has been reported in the Middle East and Sub-Saharan Africa [
3,
4]. EMT has been reported to occur more frequently in AML in association with the t (8;21) (q22; q22) or inv (16) (p 13; q22) cytogenetic abnormalities, M2, M4 or M5 morphology, CD56 positive cell phenotype and in children than in adults [
1,
2]. Iizuka Y et al. [
5] reported that AML patients in whom CD56 was expressed on the leukemic cells showed a greater incidence of development of myeloblastoma and CD56 positive AML patients should be carefully monitored for signs of myeloblastoma formation. EMT has been observed at orbit, maxilla paraspinal region, skin, subcutaneous tissue, gingiva, kidneys, ovary, testis and gastrointestinal tract [
2,
3,
6]. We observed extramedullary myeloid tumor at diagnosis as a poor prognostic factor but it should also be noted that the presence of EMT at diagnosis had no significant effect on eventfree survival because of intensive treatment regimens [
2,
3,
6,
7].