Epidemiologic studies have demonstrated increasing mortality rates from intrahepatic cholangiocarcinoma during the past decades. Primary sclerosing cholangitis is the most important predisposing condition to the development of cholangiocarcinoma. Improvements in noninvasive diagnostic techniques have led to decreased use of invasive procedures. Magnetic resonance imaging (MRI) has the potential to depict parenchymal, ductal, and vascular tumor involvement. However, diagnosis can be difficult, and often ultrasonography, MRI, CT, and invasive cholangiography are complementary investigations. Genetic aberrations in brush cytology specimens should be explored further in prospective studies. Endoscopic ultrasonography, intraductal ultrasonography, and positron emission tomography are interesting techniques that are under evaluation. Radical surgery with negative histologic margins is the only curative option in cholangiocarcinoma. With more aggressive surgical approaches, including partial hepatectomy, 3-year survival rates of 35% to 50% can be achieved. Liver transplantation for unresectable cholangiocarcinoma was shown to be feasible in pilot studies of highly selected patients.