Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), also known as Autoimmune polyendocrine syndrome type 1 (APS-1), is an autosomal recessive IEI featuring defective central tolerance, which is most often caused by biallelic deleterious variants in the autoimmune regulator (AIRE) gene [3] and characterized by multiple autoimmune manifestations in endocrine and non-endocrine organs. Here, AIRE is linked to autoimmune polyendocrine syndrome type 1.