Cholangiocarcinoma (CCA), a tumor of the bile duct epithelium, is increasing in incidence. CCA remains a highly fatal malignancy because early diagnosis is difficult. Based on its anatomical location, CCA can be categorized into the following three groups: perihilar, intrahepatic, and extrahepatic. Patients with CCA complain of asymptomatic jaundice, weight loss, and right upper quadrant abdominal discomfort. Imaging modalities, including transabdominal ultrasound, computed tomography, and magnetic resonance imaging, play an important role in detecting tumors as well as guiding biopsy procedures and staging workups in CCA. Characteristically, extrahepatic CCA shows abrupt changes in ductal diameter with upstream ductal dilation. Endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) are recommended as the next step in the evaluation of extrahepatic CCA. Tissue is obtained through EUS-FNA or ERCP (biopsy, brush cytology), and therapeutic intervention (such as stent insertion) is performed with ERCP. Moreover, several serum tumor markers (carbohydrate antigen 19-9 and carcinoembryonic antigen) can be useful in diagnosing CCA in some patients.