During the period 1989-1998, 27 patients with primary proximal bile duct cancer were treated (17 females and 10 males with a mean age of 61 years). The main presenting symptoms were: jaundice (96.2%), itching (92.5%), weight loss (74%) and atypical pain (33.3%). All the patients underwent ultrasound (US) examination, 7 patients endoscopic retrograde cholangiopancreatography (ERCP) and 4 patients computed tomography (CT) examination. US examination revealed the tumor in 51% cases; most frequently a dilatation of the intrahepatic bile ducts was diagnosed with all methods. 8 patients underwent resection of the tumor and one a biliary-enteric anastomosis, in 15 cases a stent was inserted. In 3 cases the extension of the malignancy did not allowed any procedure. Three patients died during immediate postoperative period (mortality 11.2%). The mean survival was 13 months after stenting and 22 months after resection of the tumor. The authors recommended an aggressive surgical therapy for Klatskin tumors.