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. The gene discussed is NFKB2; the disease is hyperinsulinemic hypoglycemia, familial, 4.