Carcinoma of the hepatic duct confluence is the most common site of bile duct malignancies. Significant progress has been made in recent years in the diagnosis and treatment of this disease. The diagnosis is generally made in the jaundiced patient with contrast-enhanced spiral computed tomography (CT) scanning followed by cholangiography. Percutaneous transhepatic cholangiography is favored over endoscopic retrograde cholangiography in that it better defines the proximal extent of the tumor involvement and allows placement of percutaneous transhepatic catheters. The preoperative placement of stents facilitates the surgical management, as well as decompressing the obstructed biliary tree. The operative technique at The Johns Hopkins Hospital involves resection of the hepatic confluence and reconstruction over Silastic stents. In patients in whom the hilar cholangiocarcinoma extends along either the right or left hepatic duct into the hepatic parenchyma, appropriate hepatic lobectomy is performed. A recent review of the Johns Hopkins experience has demonstrated that 67% of cholangiocarcinomas are located in the perihilar location. Fifty-six percent of these patients were resectable for potential cure. Of the 109 resected patients, 4 patients (3.6%) died secondary to complications of sepsis. In the 109 patients with resected perihilar tumors, the 1-, 3-, and 5-year survival was 68%, 30% and 11%, respectively. The median survival was 19 months. The addition of hepatic lobectomy did not alter the survival rate. Negative margins and negative lymph node status were associated with improved survival. Postoperative adjuvant radiation therapy did not provide benefit to patients with resected perihilar carcinoma.