A 78-year-old man with jaundice was diagnosed with perihilar cholangiocarcinoma(Bismuth type I ). After endoscopic biliary drainage for jaundice, a subtotal stomach-preserving pancreaticoduodenectomy was performed. Histologic examination and immunohistochemical staining with chromogranin A, synaptophysin, and CD56 resulted in a diagnosis of small cell carcinoma. Of the 18 dissected lymph nodes, 8 nodes contained a metastatic tumor. Left supraclavicular and paraaortic lymph node metastases were detected by computed tomography 5 months after the resection. He received cisplatin plus irinotecan chemotherapy, and after 2 courses of the chemotherapy, both metastatic lesions were reduced in size. He remains alive and well with no evidence of progressive disease after 6 courses of chemotherapy.