A case of cancer of the remnant pancreas after a pylorus-preserving pancreatoduodenectomy in a 72-year-old man is reported. The patient underwent a pylorus-preserving pancreatoduodenectomy (PPPD) for tubulo-villous adenoma on the minor duodenal papilla and adenomatous prolifiration on the major duodenal papilla in March 1995. In December 1999, a low density area measuring 3.5×2.5cm in the remnant pancreas was found on an abdominal CT scan. Endoscopic retrograde pancreatography showed a stenosis of the main pancreatic duct, and an abdominal angiography revealed encasement of the splenic artery and an interruption of the splenic vein. From these findings, he was diagnosed as having cancer of the remnant pancreas. In February 2000, a total pancreatectomy combined with a splenectomy and a left adrenalectomy were performed. The lesion was a nodular tumor measuring 58×48×32mm in size. Histopathological examination demonstrated moderately differentiated tubular adenocarcinoma. Conclusive stage grouping was IVa (s3, rp1, pv sp3, a sp3, pl(+), ne2 mpd(+), scirrhous type) according to the general rules of the Japanese pancreatic cancer study. Although postoperative course was uneventful, multiple liver metastasis was detected in May 2000 and he died in November 2000.