Cholangiocarcinoma is associated with several etiological factors including cystic dilation of the bile duct, clonorchiasis, hepatolithiasis, and sclerosing cholangitis. Jaundice is the presenting symptom in over 90% of patients who present with cholangiocarcinoma. The disease is suspected on the basis of an abnormal ultrasound or computed tomography (CT). Tumor markers are often normal, but a significant elevation of carcinoembryonic antigen or CA 19-9 should alert the clinician of a possible diagnosis of cholangiocarcinoma. Cholangiogram is essential to dileneate the bile duct anatomy in hilar or distal cholangiocarcinoma. A tissue diagnosis can be obtained in 60 to 70% of patients using bile cytology, brush cytology, or percutaneous fine-needle aspiration. A multidisciplinary approach is essential for optimal management. Management is based on a number of factors, including age of the patient, performance status, other comorbid conditions, location of the tumor, and tumor resectability. Complete surgical resection offers the only chance for cure in patients with cholangiocarcinoma. Tumor resectability can be accomplished using a combination of CT or magnetic resonance imaging, cholangiography, and visceral angiography. The 5-year survival rate after a potentially curative resection for hilar cholangiocarcinoma ranges from 0 to 22% (mean 14%). By comparison, the 5-year survival rate for distal cholangiocarcinoma ranges from 0 to 39% (mean 25%). Symptomatic patients who are unsuitable for curative resection can have pallitive decompression performed using either percutaneously or endoscopically placed drainage catheters.