Abstract: 　Objectives　To investigate and evaluate clinical treatment and prognosis of children with rhabdomyosarcoma (RMS). Methods　A total of 31 cases of children (19 males and 12 females) diagnosed as RMS in Children's Hospital of Nanjing Medical University from December 2009 to December 2016 were followed up, with a median age of onset at 42.2 months (7.2～109.9 months). Prognosis of patients with RMS was clinically investigated. Result　Among the 31 cases, there were 2 cases with stage Ⅰ, 6 cases with stage Ⅱ, 16 cases with stage III and 7 cases with stage IV. Protocol RS-2009 of Shanghai Children’s Medical Center was used to treat these patients until May 1, 2017. Two cases were lost to follow up during the treatment course, one case lost after replase. Median of the follow-up time is 39.3 months. After high intensive treatment, two cases with stageⅠachieved complete remission and long-term survival, two cases with stage Ⅱ and four cases with stage Ⅲ recurred, three cases with stage Ⅳ recurred and died soon, one with stage Ⅳ did not response to treatment and progressed to death. The 3-year event free survival (EFS) of the cases in stage Ⅰ, Ⅱ and Ⅲ was (61.8%±12.9%), the 3-year overall survival (OS) of them was 100%, while the 3-year EFS and OS both were (42.9±18.7%) for the cases with stage Ⅳ. The 3-year EFS of the cases with no radiotherapy was 37.7%±14.2%, while it was 82.1%±11.7% for those with regular radiotherapy. Conclusions　The protocol RS-2009 of SCMC in the treatment of RMS in stage Ⅰ, Ⅱ and Ⅲ can get a curative effect, but it need to be improved and optimized to the cases with stage Ⅳ. Radiotherapy can highly increase EFS.