PanNETs constitute a rare and heterogeneous group of pancreatic neoplasms whose overall prognosis is better than the more common pancreatic adenocarcinoma. Although surgery is the only treatment that provides a cure, many adjuvant therapies have been explored with some new, exciting, targeted therapies just approved for PanNETs. With growing interest in this type of neoplasm, an increasing number of clinical trials and natural history studies should shed light on the best management for these patients.