Primitive neoplasms of proximal extrahepatic bile ducts keep their peculiar morphological and spreading patterns, as firstly described by J. Klatskin. Diagnostic and therapeutic approach were modified in the last year, on the basis of technological progress in imaging and more aggressive surgical attitude. Authors reviewed their clinical experience from 1970 to 1995 concerning proximal extrahepatic bile ducts tumors management, mainly evaluating the evolution of diagnostic work-up and the role of resection. Preoperative work-up is now trimed to non invasive techniques, in order to evaluate the intra and extra biliary diffusion; PTC-PTBD performed preoperatively give a clear biliary map, and could be the first step of a palliative definitive treatment in case of non operable patients. Radical resection remains the gold standard of therapy, with the best long-term results. Palliation must be obtained by the easiest comfortable method for the patient (i.e. self-blocking percutaneously inserted endoprosthesis).