We report the case of a patient with a history of surgically treated pulmonary leiomyosarcoma, presenting with recurrent acute cholangitis and metastatic leiomyosarcoma of the common bile duct. Preoperative examinations had revealed a high grade malignant neoplasm and bilateral lung metastases. The patient underwent pylorus-preserving pancreaticoduodenectomy and survived for 5.5 years after the first diagnosis.