Rationale:Sarcomatoid carcinoma is an extremely rare lesion in the common bile duct (CBD).Patient concerns:We present a case of sarcomatoid carcinoma of the distal CBD in a 51-year-old woman who presented with jaundice and abdominal pain. Whipple's operation was performed successfully. Microscopically, the tumor was a poorly differentiated carcinoma containing a component of sarcoma-like differentiation. The tumor cells displayed spindle-shaped nuclei with occasional mitotic figures. Cytokeratin (CK) 7, CK19, CK18, and pan-CK (AE1/AE3) staining was positive on immunohistochemistry. Vimentin and carcinoembryonic antigen (CEA) staining were also positive.Diagnoses:Sarcomatoid carcinoma of the distal CBD.Interventions:The patient received three cycles of chemotherapy after surgery.Outcomes:The patient has experienced no adverse events in the 3 years post-surgery.Lessons:We present here a case report of sarcomatoid carcinoma of the distal CBD. The patient received chemotherapy after surgery, and was event-free for 3 years post-surgery, suggesting a relatively better prognosis, despite the infiltrative pattern of the tumor.