The safety and effectiveness of endoscopic management of ampullary adenomas is well known. However, data on the endoscopic treatment and long-term outcome of nonadenomatous ampullary neoplasms are lacking. We describe our experience with the endoscopic management and the follow-up of 4 patients with nonadenomatous ampullary neoplasms viz, ampullary carcinoid, gangliocytic paraganglioma, ampullary hamartoma in patients with Peutz-Jegher syndrome and Cowdens syndrome.