Juvenile autoimmune polyendocrinopathies are characterized by the coexistence of hypoparathyroidism, chronic candidiasis and adrenal insufficiency. This is in contrast with associated autoimmune endocrine diseases in the adult. Gonadal insufficiency is frequently encountered as well. Some other diseases can also be associated to these endocrinopathies: alopecia, diarrhea, malabsoφtion or chronic active hepatitis. The pathophysiological aspects of this syndrome are discussed.