DOI: https://doi.org/10.36347/sjmcr.2026.v14i05.058

Pages: 1112-1116

Introduction: Embryonal rhabdomyosarcoma of the biliary tree is extremely rare. It accounts for 1% of all rhabdomyosarcomas in children and 0.04% of paediatric cancers. It is often of the botryoid type and localised in the main bile duct or intrahepatic ducts. Localisation in the extrahepatic bile ducts is a rare cause of jaundice in the paediatric population. Observation: We report a case of embryonal rhabdomyosarcoma of the main bile duct managed in our hospital since 2022. Results: The patient was a 4-year-old male with cholestatic jaundice that had been progressing for 1 month and his general condition had deteriorated. Given this clinical picture, congenital dilatation of the main bile duct was suspected. Ultrasound followed by bili-MRI showed dilatation of the VBP and VBIH with echogenic material in the common hepatic duct, interpreted as a sludge. Surgical exploration revealed a distended gallbladder filled with serous fluid and endoluminal tumour-like material extending from the lower bile duct to the hepatic hilum, with no passage of bile and infiltration of the hepatic hilum, making bypass difficult. The tumour was biopsied. Histological examination was in favour of an embryonic rhabdomyosarcoma of the botryoid type. After a multidisciplinary consultation meeting in paediatric oncology, the patient received adjuvant chemotherapy and radiotherapy. The clinical course was favourable with complete regression of cholestatic jaundice. Follow-up imaging 6 months later showed total remission with disappearance of biliary tract dilatation. The patient has been followed up clinically and radiologically to date, with no sign of tumour relapse or recurrence Conclusion: Diagnostic challenges associated with Rhabdomyosarcoma of the biliary tract result from the diversity of the disease’s forms and the ambiguous clinical presentation. The standard of care and recommended treatment strategy for rhabdomyosarcoma of the biliary tract requires multimodality treatment, incl