目的　评价小儿泌尿生殖系横纹肌肉瘤不同治疗方法的疗效。方法　回顾 1972年 1月～ 1994年 12月收治的 16例小儿泌尿生殖系横纹肌肉瘤临床资料 ,前期治疗以手术 +化疗为主 ,共治疗 7例 ,其中Ⅲ期 5例 ,Ⅳ期 2例。后期治疗采用活检确诊 +冲击量化疗 +手术 +放疗的综合疗法 ,共治疗 9例 ,其中Ⅲ期 7例 ,Ⅳ期 2例。结果　前期治疗术后 5年仅存活 1例 ,后期治疗术后 5年存活 3例 ,均为Ⅲ期患儿。结论　应用活检确诊 +冲击量化疗 +手术 +放疗的综合疗法可提高Ⅲ期泌尿生殖系横纹肌肉瘤的存活率 Objective To evaluate the efficacy of different treatment of pediatric urogenital rhabdomyosarcoma. Methods The clinical data of 16 cases of pediatric urogenital rhabdomyosarcoma admitted from January 1972 to December 1994 were retrospectively reviewed. Among them, 7 cases were treated with surgery and chemotherapy, including 5 cases in stage Ⅲ and 2 cases in stage Ⅳ. Post-treatment using biopsy diagnosed + impact of chemotherapy + surgery + radiotherapy combined therapy, a total of 9 cases, of which 7 cases of stage Ⅲ, 2 cases of stage Ⅳ. Results The pretreatment only survived 5 years after operation, and 3 cases survived 5 years after the operation. All patients were in stage Ⅲ. Conclusion The biopsy diagnosed + impact chemotherapy + surgery + radiotherapy combined therapy can improve the survival rate of stage Ⅲ urogenital rhabdomyosarcoma