UDCA has a beneficial effect in PSC provided that patients who develop major duct stenoses are treated endoscopically. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. In patients with advanced disease, UDCA is not effective and liver transplantation is indicated. Since UDCA treatment leads to improvement but not to cure of cholestatic liver diseases, permanent treatment seems necessary. Such prolonged treatment with UDCA may be recommended since up to now no side effects were reported. During treatment with UDCA stenoses of major ducts may develop and early endoscopic dilatation is highly effective.