Cholangiocarcinoma (CC) is rare malignant tumors composed of cells that resemble those of the biliary tract. It is notoriously difficult to diagnose, and is associated with a high mortality. Traditionally, CC is divided into intrahepatic and extrahepatic disease according to its location within the biliary tree. Intrahepatic cholangiocellular carcinoma (IH-CCC) or peripheral cholangiocellular carcinoma (CCC) appears within the second bifurcation of hepatic bile duct, and is the second most common primary liver cancer following hepatocellular carcinoma (HCC), IH-CCC or peripheral CCC often presents with advanced clinical features, and the cause for this cancer rise is still unclear. MRI, CT and PET provide useful diagnostic information in those patients. Surgical resection is the only chance for cure, with results depending on selected patients and careful surgical technique. Liver transplantation could offer long-term survival in selected patients when combined with chemotherapy. Chemotherapy, radiation therapy or combination therapies remain as the only treatment for inoperable patients. However, these are uniformly ineffective in patients' survival.