Context: Intrapancreatic accessory spleen is an exceptionally rare entity, commonly mistaken for a pancreatic neoplasm. Preoperative diagnosis based on radiologic imaging features is difficult. Aspiration cytology may be misleading due to poor sampling.
Case report: We report a very rare case of an intrapancreatic accessory spleen which radiologically and cytologically mimicked a neuroendocrine pancreatic tumor.
Conclusion: Modern imaging techniques will probably lead to an increasing number of intrapancreatic accessory spleens being discovered, posing significant diagnostic and therapeutic challenges.