A 45-year-old woman with the diagnosis of primary sclerosing cholangitis and ulcerative colitis admitted with the complaints of pruritus and jaundice. Endoscopic retrograde cholangiography revealed entirely narrow, irregular common bile duct and common hepatic duct and unusual cystic dilations in the common hepatic duct and left hepatic duct. Balloon dilation of the common bile duct was performed, and a 10 F double pigtail stent was inserted into the cyst. Three months after the endoscopic retrograde cholangiography, cystic dilatations had completely resolved. Primary sclerosing cholangitis may present with cystic dilatations up to a level that it may resemble Caroli disease.