A 9-year-old girl was diagnosed as acute myeloid leukemia-M2 according to the French-American-British classification. In addition, a diagnosis of Turner syndrome (TS) was made, on the basis of the presence of the chromosomal abnormality, ovarian failure, and abnormal physical features. In particular, children with Down syndrome have increased risk of developing acute myeloblastic leukemia especially M7. On the other hand, cases of myeloid leukemia that are complicated with TS are extremely rare. This is the first report of TS with acute myeloid leukemia of M2 subtype and t (8; 21) in children.