Tumors of the ampulla of Vater are called ampullary tumors and can arise from any of the three epithelia (duodenal, pancreatic and biliary) that delimit the papilla. These tumors are clinically important and early identification, appropriate staging and proper treatment are essential. The symptoms of these tumors are non-specific and not always evident. All ampullary tumors must be resected but opinions differ on the optimal method of excision. Currently, controlled trials are lacking and consequently the treatment chosen must be individually tailored according to the characteristics of the patient and the tumor. Curative treatment may be endoscopic or surgical. In patients who are not candidates for curative treatment, palliative treatment through drainage can be performed.