Among pediatric panNETs, insulinomas (a type of panNET) predominate, representing 50%–60% of functional tumors and are characterized by hypoglycemic symptoms due to excessive insulin production.1) On the contrary, in all age groups, insulinomas affect 1–4 per 1 million population and are benign in 90% of cases.2) PanNETs constitute a clinically significant uncommon malignancy.1) However, insulinomas are distinct from other types of panNETs in that the majority are considered benign. The gene discussed is INS; the disease is pancreatic insulinoma.