Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) or autoimmune polyglandular syndrome type-1 (APS-1; OMIM 240300) is a monogenic disorder caused by biallelic mutations in the autoimmune regulator (AIRE) gene, a thymus-enriched transcription regulator that promotes central immune tolerance via the expression of peripheral tissue self-antigens in medullary thymic epithelial cells (1, 2). The gene discussed is AIRE; the disease is Autoimmune polyendocrinopathy type 1.