One hundred ten cases of primitive hepatic hilum neoplasms (Klatskin tumor) observed from January 1970 to June 1992 are reviewed and the variations occurring in the diagnostic back-up and treatment policy are considered. The importance of careful preoperative staging is stressed. Preoperative tests should be limited to investigations supplying most informations about endo- and esobiliary diffusion of the tumoral mass (ultrasound, direct cholangiography, portography). This paper demonstrates that a surgical approach with both palliative or resective aims is suitable for all patients with no local or general contraindications. Other cases are treated with percutaneous or endoscopic biliary stenting. The authors conclude by pointing out that tumoral resection with biliodigestive anastomosis is in any case the treatment of choice in these patients as it gives a better quality of life (improved "comfort index").