Hilar cholangiocarcinoma is a rare malignancy that occurs at the bifurcation of the bile ducts. Complete surgical excision with negative histologic margins remains the only hope for cure or long-term survival. Because of its location and proximity to the vascular inflow of the liver, surgical resection is technically difficult and may require advanced vascular reconstructions to achieve complete excision. Patients who are not candidates for resection should undergo palliative biliary drainage. The role of neoadjuvant therapy and liver transplantation in the management of hilar cholangiocarcinoma remains to be defined in light of the recent promising results.