Hyperbilirubinemias congenital - group of the hereditary enzymopathies, described disturbance of an exchange of a bilirubin and shown proof or alternating an icterus at absence of the expressed changes of structure and function of a liver, obvious attributes of a hemolysis and a cholestasia. Diseases are caused by disturbance of processes of grab by hepatocytes of a free bilirubin from a blood, its linkage with a glucuronic acid with formation of the bound bilirubin and its subsequent allocation with bile. In all cases the hyperbilirubinemia and an icterus are found out since the early childhood, in most cases - are insignificant, can have an alternating character (to amplify under influence of errors in a diet, reception of alcohol, intercurrent diseases, physical overwork and other reasons). The weak expressed dyspeptic phenomena, delicacy, fast fatigability are frequent. The liver usually is not enlarged, soft, painless, functional assays of a liver (except for a hyperbilirubinemia) are not changed. The lien is not enlarged. The osmotic resistance of erythrocytes, duration of their life - are normal.