Our recommendations are to manage asymptomatic patients with biannual clinical and biochemical follow-up examinations. Symptomatic non-cirrhotic patients who have diffuse SC should be enrolled in trials addressing the efficacy of medical therapy such as UDCA. Patients with diffuse disease and cirrhosis of the liver should be considered for liver transplantation. Symptomatic patients with a dominant stricture should first undergo rigorous investigation to rule out CCA. Disease in those noncirrhotic patients who are deemed to have benign strictures should initially be managed by means of dilation or surgical excision, although careful monitoring and review will be needed because most will continue with progressive disease, eventually showing signs of portal hypertension and cirrhosis. Patients with dominant strictures and cirrhosis should be considered for orthotopic liver transplantation. Liver transplantation in experienced units now offers more than 80% of patients a full and effective rehabilitation, with more than 75% alive at 5 yr. A progressive, advancing decompensating cholestatic disorder with an increasing risk of underlying malignancy can be stopped, giving the patient a high quality of life. More than half of patients report an improvement in the symptoms of their inflammatory bowel disease.