PT - JOURNAL ARTICLE AU - Mohammed S. Al Qahtani AU - Shoukat A. Bojal AU - Abdullah A. Alqarzaie AU - Abdulaziz A. Alqahtani TI - Insulinoma in tuberous sclerosis AID - 10.15537/smj.2021.42.3.20200490 DP - 2021 Mar 01 TA - Saudi Medical Journal PG - 332--337 VI - 42 IP - 3 4099 - http://smj.org.sa/content/42/3/332.short 4100 - http://smj.org.sa/content/42/3/332.full SO - Saudi Med J2021 Mar 01; 42 AB - 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.