Intraductal tubulopapillary neoplasm (ITPN) is a distinctive type of pancreatic tumor first discovered more than three decades ago. ITPNs currently account for less than 1% of all pancreatic exocrine tumor cases recognized, and less than 5% of pancreatic intraductal tumors. A patient's presentation is often nonspecific in comparison to other intraductal pancreatic neoplasms. We discuss a 52-year-old female presenting with abdominal pain and weight loss with ITPN, and go on to define the typical presentation, clinical features, and pathology behind the tumor.