Islet cell tumors (ICTs) are rare tumors of the pancreas. Association of this type of tumor with tuberous sclerosis is extremely rare. Only 13 cases of pancreatic ICT with tuberous sclerosis have so far been documented in the literature. However, awareness of the association of tuberous sclerosis and ICT is important for early diagnosis and appropriate treatment of this condition. This article presents the case of a 63-year-old female with angiomyolipoma (AML) of the kidney and liver, calcified subependymal nodules and a large mass in the pancreas, which was proven to be an ICT on histopathological examination.