Primary sclerosing cholangitis presenting as a localized stricture affecting a segment of the extrahepatic biliary tree is rarely found. We describe the case of a 39 year old woman with obstructive jaundice, in whom this diagnosis was proven by endoscopic retrograde cholangiography. An endoprosthesis was endoscopically introduced through the stenotic area which led to a dramatic improvement enabling a successful liver transplantation after 2 years.
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