目的：提高对小儿胆道胚胎型横纹肌肉瘤（葡萄状肉瘤）的认识，减少误诊。方法：对２例小儿胆道胚胎型横纹肌肉瘤的诊治经验教训进行分析，并复习有关文献。结果：本组例１误诊并延误治疗而死亡。例２采取积极手术并术后化疗，已健康存活８个月。结论：胆道胚胎型横纹肌肉瘤极罕见，但小儿胆系恶性肿瘤多为该种肉瘤。其恶性度高，预后差，应积极外科治疗并配合化疗、放疗，可提高长期存活率 Objective: To improve the understanding of pediatric biliary embryo rhabdomyosarcoma (grape-like sarcoma), to reduce misdiagnosis. Methods: Two cases of pediatric biliary embryo rhabdomyosarcoma diagnosis and treatment of lessons learned, and review the literature. Results: This group of cases 1 was misdiagnosed and died of treatment delay. Example 2 to take active surgery and postoperative chemotherapy, has been a healthy survival of 8 months. Conclusion: Biliary embryo rhabdomyosarcoma is extremely rare, but most of pediatric biliary malignancies are sarcomas of this kind. The high degree of malignancy, the prognosis is poor, should be actively surgical treatment and with chemotherapy, radiotherapy, can improve long-term survival