A 63-year-old woman attended our hospital after diagnosis of a solitary pulmonary metastasis from a distal bile duct carcinoma. She had undergone a subtotal stomach-preserving pancreaticoduodenectomy 1 year 9 months prior to the current presentation. She was treated with right thoracoscopic pulmonary partial resection. The specimen was a solitary nodule 5 mm in diameter. In addition, she was treated with adjuvant chemotherapy involving GEM for 6 months. One year 5 months later, she was diagnosed with a second pulmonary metastasis and was treated with a left thoracoscopic partial resection. Four months later, she experienced recurrence in the right lung stump and was treated with right thoracoscopic partial resection. The specimen was a solitary nodule 21 mm in diameter. Cytopathologic examination of the pleural fluid resulted in a diagnosis of Class Ⅴ. She was treated with adjuvant chemotherapy involving TS-1 for 6 months. Seven months later, she was experienced recurrence with multiple pulmonary metastases and an affected hilar lymph node. GEM/CDDP chemotherapy was started, but bone multiple metastases developed. After she received 30 Gy of radiotherapy, her pain improved. She died of cancer 5 years and 4 months after her initial pancreaticoduodenectomy, which was 3 years and 4 months after her initial pulmonary resection. We identified 14 other case reports of long-term survivors of distal bile duct carcinoma.