Abstract Neoplasia is an unusual complication of Meckel diverticulum. Most tumors of Meckel diverticulum are neuroendocrine or mesenchymal in origin. Adenocarcinomas represent a minority of the tumors that arise in Meckel diverticulum and are generally thought to develop from either endogenous small intestinal epithelium or heterotopic gastric epithelium. Despite the presence of ectopic pancreas in a small fraction of Meckel diverticula, convincing evidence of tumors that arise from heterotopic pancreatic exocrine tissue has not been described in this setting. Intraductal papillary mucinous neoplasms are relatively uncommon tumors of pancreatic ductal epithelial cells that line the main pancreatic duct or its major side branches. We present an unusual case of an intraductal papillary mucinous neoplasm that arose in a heterotopic pancreas within a Meckel diverticulum.