BACKGROUND:?Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by destruction and ifbrosis of the bile ducts. This study aimed to demonstrate the hepatic and extrahepatic characteristic ifndings and prognostic outcomes of Turkish patients with PSC. METHODS:?The medical records of 35 consecutive patients with PSC from January 1988 to June 2007 were recorded prospectively. From the time of diagnosis, clinical features and laboratory data were collected. RESULTS:?The mean age of the 35 patients was 41.69 years (range 15-80 years) at the time of diagnosis; 14 (40%) were female, and 21 (60%) were male. The mean duration of follow-up was 58.86 months (1-180 months). Twenty (57.1%) of the patients with PSC were asymptomatic and 22 (62.9%) had inlfammatory bowel disease. At the time of diagnosis, 20 (57.1%) of the patients had both intra- and extra-hepatic PSC. Twenty-one (60%) of the patients, who had undergone ERCP for stent placement, had dominant bile duct stenosis. Cholangiocarcinoma was found in 2 (5.7%) of the patients and cirrhosis was detected in 7 (20%); 5 (14.3%) underwent liver transplantation. The median follow-up time after liver transplantation was 23 months and all are still alive. Six (17.1%) patients died. CONCLUSIONS:?PSC has a clinical course varied from advanced liver disease requiring liver transplantation within a short time to being asymptomatic for decades. The prognosis of Turkish patients with PSC is also disappointing as described in other studies.