Background/Aims: Dominant stricture of an extrahepatic bile duct is responsible for symptoms and an exacerbation of cholestasis in 15-20% of patients with primary sclerosing cholangitis. The aim of this study was to evaluate the efficacy and safety of endoscopic treatment in this selected patient group. Methods: Retrospectively, we evaluated 16 patients who were treated endoscopically due to elevation of serum biochemical liver tests and symptoms which were attributable to dominant bile duct strictures during the period 1990 to 2003. Symptoms and biochemical liver tests were compared before and after treatment. Results: Sixteen patients underwent a total of 58 therapeutic endoscopic retrograde cholangiopancreatographies (ERCP). Sixteen endoscopic sphincterotomies, 15 balloon dilatations, 6 bougie dilatations, 3 stone / sludge extractions and 8 stentings were performed. Endoscopic therapy was technically successful in all patients (100%). Biochemical liver tests were significantly improved when compared with pretreatment values (p<0.001). Patients have been followed-up without stents except for the patients who had cholangiocarcinoma and cirrhosis at the beginning. Procedure-related early complications occurred in 8.6% of therapeutic endoscopic biliary procedures. There was no mortality due to endoscopic treatment. Two patients whose stents were changed every two to three months had cholangitis due to stenting during 13 stent periods. Four patients whose stents were changed in seven to 10 days developed suppurative cholangitis (total 6 stent periods). Conclusions: Endoscopic therapy of symptomatic dominant strictures in primary sclerosing cholangitis is safe and effective. The cholangitis seen in long-term stenting seems to be solved by short-term stenting.