Sarcomatous carcinoma of the bile duct is tremendously uncommon and aggressive. Herein, we report a case of a male who presented with jaundice. The thoraco-abdominopelvic tomography scan revealed a lesion within the common bile duct highly suspicious of malignancy. After laparoscopic pancreaticoduodenectomy, histological examination revealed a sarcomatous carcinoma. Two years after the initial diagnosis, the patient remains without signs of recurrence. Additional research about this rare condition is needed to improve care and prognosis.