Ulcerative colitis (UC) is a chronic inflammatory bowel disease (IBD) accompanied by a risk to develop colorectal dysplasia and cancer. Primary sclerosing cholangitis (PSC) is strongly associated with UC and there are some distinguishing clinical features between UC patients with PSC (UC-PSC) and UC patients without PSC. The most important feature is that UC patients with concomitant PSC are at a significantly increased risk of developing colorectal dysplasia or cancer. Furthermore, it has been suggested that patients with UC and PSC are also at an increased risk of cholangiocarcinoma. We report a case of cholangiocarcinoma and colorectal cancer diagnosed simultaneously in a patient with UC and concurrent PSC. He was diagnosed with UC and PSC 13 years ago and had been treated irregularly. Surveillance colonoscopy and biopsies were performed and one of the biopsied specimen confirmed adenocarcinoma. He underwent abdominal computed tomography (CT) because of the abnormal liver function test and the CT showed cholangiocarcinoma. To the best of our knowledge, this is the first case report of a UC-PSC with cholangiocarcinoma and colorectal cancer at the same time in Korea.