Neoplasms of extrahepatic bile ducts are rare and represent about 2% of all malignant diseases. Their clinical manifestation is delayed, when they are in advanced stage and the opportunities for radical treatment are limited. The resectability rate of the tumors of the middle and distal part of the bile ducts is higher than the percentage of the neoplasms with perihilar localization. Improved methods for preoperative diagnostic and staging as well as the individualized therapeutic approach, including biliary drainage, use of contemporary surgical techniques and methods, selective embolization of portal vein, partial hepatectomy, resection of caudal lobe, lead to increased rate of radical operations and improved long-term results.