Primary sclerosing cholangitis (PSC) is a progressive, inflammatory disease affecting extra- and intra- hepatic bile ducts with characteristic radiologic and histologic findings. PSC is frequently associated with ulcerative colitis (UC) and the incidence of cholangiocarcinoma is increased in those patients with PSC and UC. We experienced a case in which primary sclerosing cholangitis, ulcerative colitis, and peripheral cholangiocarcinoma were combined. The patient was a 37-year-old female and complained of jaundice. To our knowledge, this is the first report which has presented the association of three disease entities in Korea. (Kor J Gastroenterol 1999;34:260 - 265)