Primary biliary cirrhosis and primary sclerosing cholangitis are two most frequently encountered cholestatic condition in adults. Liver transplantation is an excellent option in patients who progress to end-stage liver disease. In these patients typical indications for liver transplantation are no different than in other conditions requiring transplantation. Liver transplantation however might be also suggested for specific indications even in patients with otherwise well preserved liver function. 5-year survival after liver transplantation in primary biliary cirrhosis was reported to be between 78 and 87%. Survival rates after liver transplantation in primary sclerosing cholangitis are considered favorable when compared to many other indications for this procedure. Nevertheless, in both primary biliary cirrhosis and primary sclerosing cholangitis recurrence of the disease after liver transplantation can be observed. Cystic fibrosis, biliary atresia, Alagille syndrome and progressive familiar intrahepatic cholestasis are cholestatic conditions in children. Liver transplantation represents an effective treatment in majority of cases fulfilling the criteria for this procedure.