Periampullary cancers are a group of tumours with similarities in symptoms and treatment, the prognoses of which are better than that of proper ductal pancreatic carcinoma. They are still sometimes diagnosed unexpectedly at operations for other reasons, such as gallstone disease and they are best diagnosed with the triad of duodenoscopy, endoscopic retrograde cholangiopancreatography and endoscopic biopsy, but one should never rely on a negative biopsy. For evaluation of resectability chest X-ray, ultrasonography and angio-CT currently are recommended for routine use and patients with resectable tumours should be offered a Whipple operation unless their general condition and/or tumour stage contradict attempted curative surgery. Long-term survival is gratifying after resection of tumours originating in the papilla, ampulla and bile duct. This is also true for those high-risk, fragile patients undergoing local excision of the tumour with free resection margins. The obstructive jaundice in patients who are pre-operatively deemed to have incurable disease or in the very frail and old patients is preferably relieved endoscopically. In those selected for laparotomy but in whom the tumour is found to be irresectable surgical biliary bypass nowadays can be done with low hospital mortality and morbidity. We feel that prophylactic gastrojejunostomy should be done selectively. If it is not possible to control pain pharmacologically palliation can often be adequately achieved by a percutaneous coeliac bloc. Additionally, the initial results of thoracoscopic sympathectomy seem promising.