A group of 36 patients, observed between 1985 and 1995, with primary carcinoma localized in the medium and distal third of biliary tract is presented. Sixteen patients (44.4%) have undergone radical surgical treatment and the other twenty (55.5%) received a palliative procedure with an average survival of 33.7 months and 8.8 months for the first and second group respectively. The actual location of the tumor was obtained by ERCP and its final stadiation occurred during operation mainly trough intraoperative ultrasonography. The preoperative biliary drainage led to a better postoperative outcome and to a better prognosis. In the case where the tumor was localized only in the third medium of the biliary tract, a limited resection of the biliary tract with Roux's reconstruction was carried out. Otherwise in the cases where the lesion was localized in the border between the medium and distal third and in the distal tract, a duodenocephalopancreasectomy was performed. Even if this is a group of lesions with a poor prognosis, when it is possible to have an early diagnosis it is necessary to carry out an aggressive surgical treatment considering the higher percentage of survival compared to any other palliative procedure.