%0 Journal Article %A Mohammed S. Al Qahtani %A Shoukat A. Bojal %A Abdullah A. Alqarzaie %A Abdulaziz A. Alqahtani %T Insulinoma in tuberous sclerosis %B An entity not to be missed %D 2021 %R 10.15537/smj.2021.42.3.20200490 %J Saudi Medical Journal %P 332-337 %V 42 %N 3 %X Pancreatic neuroendocrine tumors are rare with an incident rate of 5 cases per million individuals. Tuberous sclerosis complex is an autosomal dominant disease. This disease involves multisystem and occurs in one out of every 6,000-10,000 individuals. In this study, we describe a 47-year-old male known tuberous sclerosis patient with an insulinoma. The tumor was incidentally detected in follow-up imaging for a previous ampulla of Vater tubular adenoma. However, the patient reported symptoms of hypoglycemia. The insulinoma was enucleated successfully. Histopathology revealed a well-differentiated, grade one neuroendocrine tumor measuring around 2 cm in diameter. Seven cases were reported in the literature of tuberous sclerosis-associated insulinoma. The 7 reported cases had different hypoglycemia related symptoms. The reported tumors varied in size and location on the pancreas. This paper details the eighth case worldwide where an insulinoma occurred in a tuberous sclerosis patient. %U https://smj.org.sa/content/smj/42/3/332.full.pdf