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This article presents a review and a case report of autoimmune polyglandular syndrome type 1 (APS-1) in a child with autoimmune hepatitis (AIH) as a first clinical manifestation. The duration of the disease was 9 years. The first signs of hepatitis (jaundice, hepatosplenomegaly, impaired pigment metabolism, cytolysis) with a high degree of activity and a morphological picture of monolobular liver cirrhosis with stromal and parenchymal activity were noted at the age of 2.5 years. The child received therapy with prednisolone. After one year, symptoms not typical of AIH were noted: salting food, candidiasis of the nail plates on hands and feet. Upon repeated examination, the diagnosis of APS-1 (hypoparathyroidism, candidiasis, autoimmune thyroiditis, chronic adrenal insufficiency, autoimmune hepatitis) was confirmed genetically – the homozygous R257X mutation was detected. The therapy was corrected: fludrocortisone and diflucan were added, therapy was continued with gradual transition to a maintenance dose of prednisolone. This case report demonstrates the difficulty of early APS-1 diagnosis, resulting in late initiation of baseline therapy, which can determine the prognosis of the disease. Key words: children, autoimmune polyglandular syndrome, autoimmune hepatitis