Pancreatic neuroendocrine tumors (PNETs) account for 1 to 2% of all pancreatic tumors and most of them are sporadic and non-functioning, 5–7% arise within inherited syndromes, including MEN1, Von-Hippel Lindau (VHL) syndrome, neurofibromatosis type 1 (NF1), and tuberous sclerosis. The gene discussed is MEN1; the disease is von Hippel-Lindau disease.