Even though autoimmune polyendocrine syndrome 1 (APS-1) represents the paradigm of the immune disorder associated with endocrinopathy, other disorders such as immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, CTLA4 deficiency, LRBA deficiency, STAT1 gain of function (GOF), NFKB2 LOF, STAT3 GOF must be considered. Here, CTLA4 is linked to hyperinsulinemic hypoglycemia, familial, 4.