Cholangiocarcinoma (CCA), stratified into intrahepatic cholangiocarcinoma (iCCA) and extrahepatic cholangiocarcinoma (eCCA), is a rare malignancy, accounting for 15% of all primary liver cancers and 3% of all gastrointestinal cancers. Based on data from the NCI Surveillance, Epidemiology, and End Results Program (SEER), localized disease carries a 5-year survival rate of 23%, which drops to 9% with regional disease, and 3% for distant disease. In this chapter, the epidemiology, pathophysiology, diagnosis, staging, and management of intrahepatic cholangiocarcinoma are discussed.