Patients with Stage IV cholangiocarcinoma are currently not considered to be surgical candidates and are typically offered systemic chemotherapy. Recently, several novel systemic chemotherapy regimens have allowed an initially unresectable cholangiocarcinoma to be resectable. The aim of this article is to present the usefulness of adjuvant surgery in a case of advanced cholangiocarcinoma that was successfully treated with gemcitabine. A 72-year-old man was diagnosed with distal cholangiocarcinoma with liver metastases (cT2N0M1, Stage IV). He underwent metal stent placement in the duodenum to alleviate jaundice. After 18 courses of chemotherapy using gemcitabine without severe drug toxicities, a computed tomography scan showed that the liver metastases in S6 and S7 had disappeared. The patient underwent subtotal stomach-preserving pancreaticoduodenectomy and lymph node dissection. The pathological stage was pT2N0M0, Stage IB. The patient underwent 6 cycles of adjuvant chemotherapy using gemcitabine. The patient is alive and well 6 years and 9 mo after the diagnosis.