Cholangiocarcinoma is a primitive cancer of the bile ducts due to the transformation of the cells that constitute the epithelium of the bile ducts (cholangiocytes). This tumor is classified according to its localization and may be intraepathic, that is, it originates within the liver, or extraepathy, which is formed in the bile ducts and is then subdivided into hilar and distal. Risk factors favoring the development of cholangiocarcinoma are: primary cirrhosis, hepatitis, primitive sclerosing colangitis, biliary cysts, parasitic infections, liver and metabolic syndrome. Extrahepatic cholangiocarcinoma generally occurs with jaundice, while the symptoms of intraepathic colangiocarcinoma are less defined and specific. This tumor is often treated surgically by liver resection.