Authors describe in the case report a development of the primary hemochromatosis. At the time of the diagnosis in 1979 from organ complication the liver fibrosis and secondary diabetes mellitus were presented. Patient was treated with the venepunctions and with desferoxamine. In the 1998 the primary hepatocellular tumor was found and resected. Instead of the chemotherapy occurred the progression of the disease with multiple metastases and liver insufficiency and 66-years old patient died 19 months after surgery in the 20th year of the disease.

        Key words: The primary hemochromatosis - Mutant gene of hemochromatosis (HFE) - Ferritin - Alpha-fetoprotein - Hepatocellular carcinoma