Rhabdomyosarcoma of the biliary tree

Rhabdomyosarcoma (RMS) of the biliary tree is a rare tumor in children that has a very poor prognosis. Preoperatively, it is often mistaken for a choledochal cyst. We report a case of RMS of the biliary tree in a 4-year-old girl who presented with abdominal pain and obstructive jaundice. The RMS was diagnosed at laparotomy; excision was not possible due to its size and localization. Chemotherapy achieved complete regression of the tumor observed at second-look surgery. Preoperative chemotherapy can now avoid mutilating surgical procedures and improve survival.