Abstract: Objective　To explore the clinical manifestations and prognosis of pediatric acute megakaryoblastic leukemia (AMKL). Methods　The clinical data of 22 patients admitted for AMKL from February 2011 to August 2017 was retrospectively analyzed. Survival analysis were estimated by Kaplan-Meier method and Log-Rank test. Results　The median follow-up time of the 22 patients was 10.8 months, in which 4 cases gave up after diagnosis and 3 cases abandoned treatment in the first course of induction chemotherapy. The total complete remission (CR) rate of the remaining 15 patients was 66.7% and 93.3% after the first and second chemotherapy course, respectively. Marrow relapses occurred in 8 patients, with the 2-year OS and EFS rates of 36.4%±13.7% and 22.2%±10.7% , respectively. Univariate analysis showed that the 2-year OS rate of patients with acquired trisomy 21 was significantly higher than that in the other patients (P=0.019) and the 2-year EFS rate of patients with chromosome number between 49 to 60 was significantly higher than that in the others (P=0.028). The 2-year OS and EFS rates of patients with initial white blood cell count below 10×109/L were higher than those above 10×109/L (P=0.047, P=0.04) and the 2-year OS and EFS rates of patients who received allogeneic hematopoietic stem cell transplantation (HSCT) in first CR were higher than those receiving chemotherapy alone (P＜0.05). Conclusions　AMKL is a highly heterogeneous disease, with a high recurrence rate and a poor prognosis. Initial white blood cell count and karyotype are important factors affecting the long-term therapeutic effect. HSCT should be applied as soon as possible after CR.