BACKGROUND Symptomatic biliary obstruction caused by cavernous transformation of the portal vein is an extremely rare disorder for which there is no consensus as to optimal treatment. The results of endoscopic treatments in a small group of patients is reviewed. METHODS A total of 10 patients (5 men, 5 women; mean age 36.1 years, range 17-48 years) with severe biliary strictures were treated between 1995 and 2001. Biliary sphincterotomy was performed in all patients. Four patients also underwent balloon dilation, nasobiliary drainage, and stone or sludge extraction by using a balloon. All patients had stent insertion. METHODS The mean duration of therapy was 3.3 years (range 1-7 years). There was no complication directly related to the endoscopic procedures except for hemobilia that occurred in one patient during stent removal. Cholangitis developed in 5 patients during the therapy period and was treated endoscopically. In 4 patients, significant improvement in the biliary stricture was observed and stents were removed in 3. These patients were followed without stent insertion for one year. CONCLUSIONS Endoscopic management of biliary stricture caused by cavernous transformation of the portal vein appears to be effective and safe.