Biliary rhabdomyosarcoma (RMS) is a rare malignancy of childhood. The radiological findings and clinical presentation of the tumor can mimic an entirely different pathology. The incidence of RMS has impeded the development of a standardized form of treatment. A four-year-old child with botryoid RMS in the common bile duct is reported herein to emphasize the role of surgery in the small-sized tumor and the role of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis and relief of biliary obstruction before total excision.