Liver transplantation is the therapy of choice in the final stage of PBC, PSC and biliary atresia. UDCA leads to remission of the symptoms and the clinic in the early stages not only of these diseases but of cholestasis of pregnancy and of cholestasis in cystic fibrosis as well. In benign intermittent cholestasis only phenobarbital but not UDCA seems to be efficacious. The efficacy of UDCA in chronic active hepatitis and alcoholic hepatitis is not yet guaranteed.