Cholangiocarcinoma is a primary hepatic malignancy originating from bile duct epithelium. It is the second most common primary hepatic neoplasia, and its incidence has increased within the last 3 decades. Although several risk factors have been identified, especially chronic biliary tract inflammation, most patients with cholangiocarcinoma have no identifiable risk factors. Recent developments in radiologic and molecular diagnostic methods have helped in the diagnosis of this disease. The only curative therapy is surgical resection or liver transplantation. For patients with advanced stage disease, survival remains limited. With growing understanding of the molecular and cellular etiology of this disease, new targeted therapies are being developed.