The authors report eight cases of biliary duct rhabdomyosarcoma in children, examined by US and CT. There were five boys and three girls, aged 2 to 17 years. At presentation, US demonstrates the tumor mass within the liver or the hepatic hilum; it allows measurement of it and defines the relationship with portal vessels, biliary tract and other important structures. CT complements the US evaluation and determines operability. As US and CT cannot assess the histological origin of the tumor, a biopsy is mandatory before treatment. If complete surgical excision does not seem possible, percutaneous biopsy is preferable to incomplete excision and its possible complications. During the follow-up period, US can be repeated to measure tumor regression under chemotherapy. After surgery, CT seems preferable because of gas interposition. Both US and CT proved to be valuable for the early detection of local recurrence. The prognosis of these tumors remains bad. However, with more aggressive and hopefully more efficient chemotherapy a precise evaluation of the tumor extension by US and CT is very important. Surgery will then be performed only on localized tumors or on residual masses after chemotherapy.