Autoimmune polyendocrinopathy syndrome (APS)is a multiple endocrine dysfunction caused by autoimmunity disorders. It can commonly be divided into two types,including APS type Ⅰ and APS type Ⅱ. There are significant differences in composition and pathogenesis of these two types. APS Ⅰ is caused by mutations of the autoimmune regulatory (AIRE) gene on chromosome 21 and genetic screening may identify patients with APS Ⅰ. For APS Ⅱ , the susceptibility are often related with human lymphocyte antigen (HLA) genes which only indicated the increased risk for developing autoimmune disorders. The occurence of APS Ⅱ is also influenced by many other factors. Here discusses the epidemiology, etiological factors,pathogenesis, clinical signs and symptoms and treatment of APS, so as to enhance the screening and follow-up of APS in clinical investigations.