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Background. Autoimmune hepatitis is a chronic inflammatory disease of the liver of unknown etiology, characterized by the loss of tolerance against hepatic tissue, leading to the destruction of hepatic parenchyma. It predominantly affects females, and rarely occurs in the same family. Case report. In this paper we presented brother and sister with autoimmune hepatitis according to the criteria of the International Autoimmune Hepatitis Group. Conclusion. Because of a possible genetic predisposition to autoimmune hepatitis, the occurrence of the disease in a family member suggested the need to examine other family members.