We report the case of a periampullary collision tumor, in which a duodenal-wall carcinoid and an adenocarcinoma of the head of the pancreas coexisted. We describe the case of a 64-year-old man with a recent history of diarrhea, who was diagnosed with obstructive jaundice. A duodeno-pancreatectomy was performed, and the specimen showed two independent neoplasms in the histopathologic study. Solid cords and nests of neuroendocrine cells in the duodenal wall formed the carcinoid tumor, whereas the other neoplasm was made up of a well-differentiated adenocarcinoma of the pancreas. Both were confirmed by immunohistochemical analysis. According to the literature reviewed, this is the sixth reported case of this rare neoplastic association.