Ectopic pancreas is a commonly seen congenital anomaly in gastrointestinal system, but jejunal pancreatic heterotopia is seen rarely. Although every pathological change that occurs in the pancreas can be seen in its heterotopic counterpart, neoplasia is an unusual complication. Intraductal papillary mucinous neoplasms (IPMNs) are tumors originate from ductal epithelial cells of the pancreas that constitute the main pancreatic duct or its major side branches and have a low incidence. Only two cases regarding the IPMN in heterotopic pancreas have been reported previously, but none of them were located in the jejunum. An IPMN in an ectopic pancreas tissue located in the jejunum in a 62-year-old male patient who presented with ileus is presented. It is important to recognize a tumor arising in heterotopic pancreas in order to prevent its misinterpretation as metastatic tumor or direct invasion of another tumor. To the best of our knowledge, this is the first reported IPMN case occurring in ectopic pancreas tissue located in the jejunum.