Since the description by Klatskin in 1965, the management of patients with adenocarcinoma of the hepatic bile duct bifurcation is viewed as a challenging clinical problem with a relatively poor prognosis. Surgery continues to be the mainstay of therapy. Complete resection of the tumor with negative histologic margins offers the best possibility of long-term survival, and hepatic resection is a critical component of the operative approach. Adjuvant chemoradiotherapy has failed to provide a significant survival benefit. Orthotopic liver transplantation for otherwise unresectable lesions remains controversial, as tumor recurrence has been reported in more than 90% of patients. With the shortage of organs, such patients to be selected carefully for transplanation. For patients who present with widespread disease and those with high operative risks, advances in interventional radiology and endoscopy have facilitated nonsurgical management options. Biliary decompression using expandable metallic stents provides superior patency and decreased frequency of hospitalization when compared with plastic stents. Moreover, patients treated with expandable metal stents have survival rates comparable with those who undergo surgical decompression, with fewer early complications. The benefit of external beam radiotherapy for palliation of proximal cholangiocarcinoma is uncertain. Radiotherapy in conjunction with biliary stenting has a survival benefit over stenting alone, but is not without potential toxicity. It should be considered as an adjunct to biliary decompression in all patients with good performance status, because modern conformal CT-based dosimetry can minimize toxicity to normal adjacent tissue. Photodynamic therapy is emerging as a new palliative treatment modality for patients with unresectable tumors in whom stenting has failed. It offers the advantage of an endoscopic delivery system, and unlike radiotherapy, photodynamic therapy may be delivered repeatedly.