The registry of digestive tract tumours established for the department of Cote-d'Or (France) was used to study the epidemiological characteristics and the natural history of biliary tract cancers. Age standardised incidence rates for gallbladder cancers were 2.7/100,000 for women and 0.9/100,000 for men. The corresponding rates for extrahepatic bile duct cancers were 0.5/100,000 and 1.7/100,000, and for ampulla of Vater cancer 0.3/100,000 and 0.3/100,000. The three cancers differ in their descriptive epidemiology and should be considered separately in epidemiological analytical investigations. The incidence of each of the three diseases increased with age, and cancers of known histological type were mainly adenocarcinomas. Some gallbladder cancers were undifferentiated or squamous cell carcinomas. There was no significant variation in incidence for gallbladder cancer and extrahepatic bile duct cancer over the eight years of the study. The association with gallstones was frequent in gallbladder cancer: 70.5% compared to 13.0% in other biliary tract cancers (less than 0.001). Although the association of gallbladder cancer with gallstones is frequent, few patients with cholelithiasis experience development of a gallbladder cancer. It is necessary to identify among patients with gallstones a subgroup at high risk of gallbladder cancer in whom prophylactic surgery might be justified. Biliary tract cancers are seldom diagnosed early: lymph nodes or visceral metastases were present in 77% of gallbladder cancers, in 83% of extrahepatic bile duct cancers, and in 55% of ampulla of Vater cancers at the time of diagnosis. The corresponding resectability rates were 46.1%, 11.9%, and 38.9%. The five-year overall survival rates were 2.9% for gallbladder cancer, 0% for extrahepatic bile duct cancer, and 18.3% for ampulla of Vater cancer. The corresponding five-year survival rates after surgery for cure were 10.3%, 0%, and 35.7%. Biliary tract cancer still represent a great therapeutic challenge.