Abstract: Objective Clinical features of 12 cases of primary sclerosing cholangitis (PSC) , a rare disease in China, were analyzed in order to improve the recognition of the disease here.Methods 12 cases of PSC identified by radiological and/ or pathological examinations from November 2005 to June 2009 were analysed retrospectively.Results Of 12 patients, 8 cases were female and 4 cases were male.The mean age of onset was 43.8 years old.The majority of PSC patients presented with jaundice, fatigue, pruritus, anorexia, and splenomegaly.Elevation of alkaline phosphatase (ALP) and r-glutamyl transpeptidase (GGT) occured in 100% of cases, and a mild to moderate elevation of aminotransferase levels was also pres-ented.Bilirubin levels fluctuated.Antinuclear antibody (ANA) was positive in 7 cases.Antineutrophil cytoplasmic antibody (ANCA) was positive in 5 cases.The immuno-globulin G level was high in 8 cases.7 of 8 cases who had a liver biopsy had the typical findings of PSC.After therapy with ursodeoxycholic acid (UDCA) , 9 cases had improvements in biochemi-cal test, 2 cases of cirrhosis and 1 case of cholangiocarcinoma had no effect.Conclusion The patients with unknown causes cholestasis and elevated ALP and GGT should exclude PSC, magnetic resonance cholangiopancreaticography (MRCP) , endo-scopic retrograde cholangiopancreaticography (ERCP) or liver biopsy examination should be carried out.