Autoimmune polyendocrine syndrome type-1 (APS-1), also known as polyendocrinopathy-candidiasis-ectodermal dystrophy or APECED, is a monogenic inborn error of immunity (IEI) caused by mono- or biallelic loss-of-function variants of the autoimmune regulator gene AIRE [1–4]. Here, AIRE is linked to autoimmune polyendocrine syndrome type 1.