【摘要】 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，可提高长期存活率更多还原

【Abstract】 Objective: To improve the understanding and diagnosis of embryonal rhabdomyosarcoma of biliary tree in childhood. Methods: The management of 2 children with embryonal rhabdomyosarcoma of the biliary tree was reviewed. Results: The first child died two months after diagnosis due to delay in both diagnosis and treatment. The second child is alive and well after surgical intervention and chemotheraphy. Conclusions: Embryonal rhabdomyosarcoma of the biliary tree, though rare, is the most common cause of biliary obstruction due to malignancy in childhood. A combined approach of aggresstive surgery, chemotherapy and radiation may result in longterm survival.更多还原