Objective:To improve the understanding and diagnosis of embryonal rhabdomysarcoma of biliary tree in childhood.Methods:The management of 1 child with sarcoma botryoides of biliary tree and other 15 cases reported were reviewed.Results:11 cases of boys and 5 of girls

varying from 1 to 6 years old.Clinical symptoms at presentation included jaundice in 16/16

fever 8/16

abdominal pain 6/16

white feces 9/16 and hepatomegaly 13/16

etc.5 cases of which were misdiagnosed as having hepatitis.There were only 2 cases considered as rhabdomyosarcoma by ultrasound and 1 by abdominal CT scan.7 cases had not recurred in 8 months after combined treatment of complete surgical excision

chemotherapy and radiotherapy.Treated with combination of partial excision and chemotherapy

1 case’s tumor turned smaller 3 months later.But 2 cases treated solely with partial excision died 2 months later and 1 case recurred 2 months later.Conclusion:All rhabdomyosarcoma of biliary tree in childhood are botryoid

embryonal rhabdomyosarcoma.The patients are aged from 1 to 6 years old

and boys are more common than girls.The main clinical symptoms are obstructive jaundice

fever and hepatomegaly

so it is easily misdiagnosed as hepatitis in clinic.Ultrasound and abdominal CT sacn are helpful in diagnosing embryonal rhabdomysarcoma (sarcoma botryoides) of biliary tree

but pathological examination is essential for confirming the disease.A combined approach of aggressive surgery

chemotherapy and radiotherapy may result in long-term survival.