Monogenic autoimmune polyendocrine syndrome type 1 (APS-1), or autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), caused by AIRE mutations, exemplifies a lifelong predisposition to multiple endocrine autoimmune targets, notably hypoparathyroidism and Addison’s disease. Here, AIRE is linked to hypoparathyroidism.