The article reviews the current definition, diagnosis and treatment of hereditary hemochromatosis. The scre-ening examination includes transferrin saturation and sérum ferritin. It is indicated in patients with crypto-genic liver disease, arthropathy, diabetes mellitus or impairment of glucose tolerance, unclear arrhythmia or cardiomyopaťhy. Genetic examination offers finál confirmation of the disease. Treatment with venepuncti-on is simple and most effective. Early diagnosis and treatment allows the patient a normál life expectancy

        Key words: hemochromatosis, ferritin, transferrin saturation, iron, diagnosis, treatment.