A 59-year-old man was diagnosed as having a pancreatic carcinoma with synchronous liver metastasis at initial surgery. After wedge resection of liver tumor for histopathological analysis and gastro-jejunostomy, he was treated with 3 cycles of combined systematic chemotherapy consisting of CDDP and CPT-11, because of histopathological diagnosis confirming a neuroendocrine carcinoma of the pancreas. After chemotherapy, there was no recurrence and the primary tumor was reduced in size. Therefore, pancreatico-duodenectomy was performed as a curative treatment in two stages. During the follow-up, the patient has been alive without any signs of recurrence for 20 months since the diagnosis. Recently, several consecutive chemotherapies have been an effective modality to improve a poor prognosis for unresectable neuroendocrine carcinoma.