Researches have shown that the appearance of this chromosomal abnormality was often associated with therapy-related myelodysplastic syndrome, therapy-related acute myeloid leukemia,[1,2] the blastic crisis phase of chronic myelogenous leukemia,[1,3,4] and on rare occasions, de novo AML.[5] We here report the clinical manifestations, laboratory findings, and therapeutic outcome of a little girl diagnosed as de novo acute myelomonocytic leukemia (AML-M4) with RUNX1-MECOM and RUNX1-RPL22 fusion genes. This evidence concerns the gene RUNX1 and myelodysplastic syndrome.