Objective: Intraductal papillary mucinous neoplasms (IPMNs) are a recently classified pancreatic neoplasm with an increasing incidence. IPMN of the pancreas is a lesion consisting of mucin-producing cells with neoplasic potential, characterized by papillary intraductal growth and varying degrees of cytologic atypia. Neuroendocrine tumors of pancreas (PNET) are rare pancreatic neoplasms comprising 1-2 % of all pancreatic tumors. PNETs express neuroendocrine markers, however the true cell or cells of origin are not fully understood. PNETs are classified as functional or nonfunctional based on the presence or absence of a specific clinical syndrome associated with hormone oversecretion. Intraductal papillary mucinous neoplasm of the pancreas with concomitant Neuroendocrine tumor are a highly rare occurrence. Method: A literature review of IPMNs and PNET was made, as well as a review of the clinical data of a patient with IPMN and concomitant PNET. Results: A 60-years-old Caucasian female, underwent a laparoscopic distal pancreatectomy with splenectomy for IPMN. Macroscopic examination revealed discrete dilatation of pancreatic ducts and nodule in the pancreas tail. Hystologic examination revealed neoplasic lesion of intraductal papillary-mucinous in the body and tail of pancreas with moderate dysplasia (IPMN borderline) and Neuroendocrine Tumor grade 1 (WHO 2010). Conclusion: This case reports a highly rare occurrence.