Intraductal papillary mucinous neoplasia (IPMN) is a comparatively recently recognized pancreatic neoplasm with considerable malignant potential. Surgical removal is the only therapy known to provide a cure, but the extent of surgery required is still a matter of discussion. Ectopic pancreatic tissue can occur in a variety of other locations within the gastrointestinal tract and is also known to harbor pathologic conditions that affect normally located pancreatic tissue. In this paper, we present a case of an intraductal papillary mucinous carcinoma arising within the ectopic pancreatic tissue in the wall of the proximal duodenum. The lesion was removed by a laparoscopic resection. We believe this to be the first described case of a malignant IPMN to arise within ectopic pancreatic tissue and also the first report of the laparoscopic removal of any ectopic pancreatic IPMN.