Autoimmune polyendocrine syndrome type 1 (APS1) is a rare autosomal recessive disorder(3) caused by mutations in the autoimmune regulator (AIRE) gene(4,5) and is characterized by multiple organ-specific autoimmunity and ectodermal manifestations.(3,6) In the majority of cases, disease components include mucocutaneous candidiasis, hypoparathyroidism, and Addison's disease, with type 1 diabetes mellitus, alopecia, vitiligo, autoimmune hepatitis, and pernicious anemia occurring less frequently. The gene discussed is AIRE; the disease is primary adrenal insufficiency.