The paper gives an update on the pathogenesis and main clinical manifestations of primary (hereditary) hemochromatosis and its diagnostic methods. It emphasizes the importance of genetic research in the diagnosis of the disease. Its clinical manifestations are associated with iron deposition in organs, such as the liver, pancreas, joints, skin, and heart. The paper points out the importance of liver biopsy using Perls’ reaction for the diagnosis of liver damage with the development of fibrosis, cirrhosis, and cancer as the main cause of death in patients with hereditary hemochromatosis and presents the results of examining liver biopsy specimens in patients with hemochromatosis.