Primary sclerosing cholangitis is a chronic disease of unknown cause, characterized by inflammation and fibrosis of the biliary tree with diffuse multifocal stricture formation. With increasing knowledge of primary sclerosing cholangitis, it is now recognized that in the setting of inflammatory bowel disease, cholangiocarcinoma is a complication of primary sclerosing cholangitis. We recently experienced a case of 41 year old female patient who had Crohns disease associated with primary sclerosing cholangitis and cholangioearcinoma. We report a case of primary sclerosing cholangitis with cholangiocarcinoma with literature review. (Korean J Gastrointest Endosc 17: 99~104, 1997)