Cholangiocarcinoma is the second most common primary malignant tumor in the liver. It is a tumor that is characteristically composed of cells resembling those from the bile duct. The disease is difficult to diagnose and is usually fatal due to its late clinical presentation, lack of effective non-operative therapy, and rapid turnover. Most patients have unresectable tumors at the time of presentation and die within 12 months once diagnosis has been made. Prognosis of intrahepatic cholangiocarcinoma (ICC) remains very poor. Currently, there is no established therapy once diagnosis is made. In this report, we provide a case of a patient who presented with ICC and positive history of hepatitis C virus (HCV). The patient also had a strong family history of cancer. Finally, we attempt to review some of the important developments in the study of ICC, with particular attention to recent studies linking hepatitis with the disease.