Despite the overall advances in the ability to diagnose and treat patients with perihilar cholangiocarcinoma, the prognosis for patients with this malignancy remains poor. Further improvements in the survival of patients with perihilar cholangiocarcinoma will come with the early diagnosis of these lesions. New molecular techniques should improve our ability to screen high-risk patients, such as those with primary sclerosing cholangitis, hepatolithiasis, choledochal cysts, and ulcerative colitis. Improvements in diagnostic CT scanning, duplex ultrasonography, and MRI will enhance our ability to noninvasively stage patients with perihilar cholangiocarcinoma. Complete surgical resection remains the only curative treatment for malignancies of the biliary tract. Aggressive surgical approaches are likely to continue, and the challenge remains being able to perform these procedures safely in jaundiced and sometimes septic patients. For those patients with unresectable lesions, the optimal form of palliation, whether operative or nonoperative, remains to be defined. Finally, advances in adjuvant chemotherapy and radiotherapy will be required to further improve the overall prognosis of patients with perihilar cholangiocarcinoma.