Objective To investigate risk factors which affect the prognosis of children with rhabdomyosarcoma (RMS). Methods A total of 56 cases of RMS were evaluated in a retrospective analysis.Results In univariate analysis, tumor' s primary location and size, pathologic type, clinical stage,operation effect and therapeutic strategy were associated with prognosis of children with RMS (P <0.05). In multivariate analysis, clinical stage was an independent prognostic factor of RMS (x 2 = 8.762, P=0.003).Conclusion Tumor ' s primary location and size, pathologic type ,clinical stage, operation effect and therapeutic strategy are associated with the survival of children with RMS. Clinical stage is an independent prognostic factor of RMS. The therapeutic strategy combined with operation, adjuvant chemotherapy and radiotherapy can increase the survival rate.