Ulcerative colitis (UC) is a severe disease of the gastrointestinal tract characterized not only by local complications but also by frequent extraintestinal manifestations. This paper is designed to report a case of UC  affecting a 45 year-old woman (patient B.) complicated by the development of colorectal cancer and extraintestinal clinical symptoms in the form of primary sclerosing cholangitis followed by the formation of  a rare variant of cholangiocarcinoma known as Klatskin tumour.