AIRE and autoimmune polyendocrine syndrome type 1: <h4>Background</h4>Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a rare monogenetic autosomal recessive disorder caused by a mutation in the autoimmune regulator (<i>AIRE</i>) gene characterized by complex phenotypic characteristics discovered over years of follow-up.<h4>Methods</h4>7 patients were recruited in this case series in a period of the last 37 years from Southern Croatia.