This case report describes the unusual presentation of a patient who had findings which were initially suggestive of a typeⅣcholedochal cyst.Her liver biopsy demonstrated biliary cirrhosis.She was treated with endoscopic retrograde cholangiopancreatography and biliary stent exchanges over one year.Her cholangiogram one year later demonstrated resolution of the biliary cystic dilation which led to her initial diagnosis,with beading and stricturing of the hepatic ducts consistent with primary sclerosing cholangitis. Liver-associated enzymes and physical findings also improved.A liver biopsy one year later demonstrated a marked improvement in hepatic fibrosis with no evidence of cirrhosis. This case report describes the unusual presentation of a patient who had findings which were initially suggestive of a type IVcholedochal cyst. Her liver biopsy demonstrated biliary cirrhosis. He was treated with endoscopic retrograde cholangiopancreatography and biliary stent exchanges over one year. resolution of the biliary cystic dilation which led to her initial diagnosis, with beading and stricturing of the hepatic ducts consistent with primary sclerosing cholangitis. Liver-associated enzymes and physical phenomena also improved. A liver biopsy one year later demonstrated a marked improvement in hepatic fibrosis with no evidence of cirrhosis.