目的　探讨胎儿横纹肌瘤型肾母细胞瘤 (FRN)特点和恰当的治疗方法。方法　从195 5～ 1996年 36 9例肾母细胞瘤中检出FRN 10例。回顾性分析其临床表现、病理组织学特点和预后。结果　 10例FRN均因腹部肿大或腹部肿物入院。经术前放、化疗肿瘤不缩小。 6例单侧病变做瘤肾切除 ,5例获随访均长期存活。 4例双侧病变中 1例做双侧单纯肿瘤切除 ,3例做一侧瘤肾切除 ,对侧肾部分切除或单纯肿瘤切除 ,随访存活 2例。结论　FRN是一种少见的特殊类型的肾母细胞瘤。对术前放、化疗不敏感 ,但侵袭性小 ,较少发生转移 ,应按预后好的组织结构化疗。对双侧病变单纯肿瘤切除可更多的保留肾组织 ,提高生存质量。 Objective To investigate the characteristics and appropriate treatment of fetal rhabdomyosarcoma nephroblastoma (FRN). Methods From 1959 to 1996, 36 cases of nephroblastoma were detected in 10 cases of FRN. Retrospective analysis of its clinical manifestations, histopathological features and prognosis. Results All 10 cases of FRN were admitted to the hospital due to abdominal enlargement or abdominal mass. After preoperative radiotherapy, chemotherapy does not shrink the tumor. 6 cases of unilateral neoplasia nephrectomy, 5 cases were followed up long-term survival. In 4 cases, bilateral nephrectomy was performed in 1 case, bilateral nephrectomy was performed in 3 cases, partial nephrectomy was performed in 3 cases, and 2 cases were followed up. Conclusion FRN is a rare and special type of nephroblastoma. Preoperative radiotherapy and chemotherapy is not sensitive, but less invasive, less metastasis, should be good prognosis of the organizational structure of chemotherapy. Simple bilateral tumor resection can retain more kidney tissue, improve quality of life.