A 52-year-old male was presented with obstructive jaundice and liver dysfunction. He was diagnosed as hilar cholangiocarcinoma involving the confluence of the right and left hepatic duct and bifurcation of the main portal vein trunk. Swollen lymph nodes in the hepatoduodenal ligament were also detected. ERBD tubes were placed in each B2, 3, and 5 branch. GEM and S-1 combination chemotherapy was carried out for four months. As a reduction in the primary tumor and lymph nodes was observed on CT scan surgical exploration was conducted, and an extended left hepatectomy with partial resection of the portal vein and regional lymph node dissection was achieved. The postoperative course was uneventful, and the patient remained free of recurrence, 34 months after the original diagnosis was made, and 29 months after surgical resection. Thus, GEM and S-1 combination chemotherapy is one of the options for the management of advanced hilar cholangiocarcinoma.