A male in his twentieth was referred to our hospital for jaundice. Computed tomography(CT)showed dilation of the intrahepatic and extrahepatic bile ducts and showed a lesion at the ampulla of Vater, which caused obstructive jaundice. Upper gastrointestinal endoscopy revealed a tumor of protruded-predominant type with raised margins at the ampulla of Vater, and biopsy from the lesion indicated malignancy. With no apparent distant metastasis, radical resection was assumed to be possible, thus we performed subtotal stomach preserved pancreatoduodenectomy. Before the operation, endoscopic retrograde biliary drainage(ERBD)was unsuccessful because of the existence of the tumor, so percutaneous transhepatic cholangio drainage(PTCD)was conducted. After the operation, although pancreatic fistula(ISGPF Grade B)occurred, it improved with conservative treatment, and he discharged at 30 postoperative days. Histopathological examination revealed signet-ring cell carcinoma among the tumor at the ampulla of Vater, which was infiltrating into the pancreas. Final diagnosis was pT3, pN0, M0, pStage ⅡA. Now he is alive without recurrence for 3 and a half years.