Gangliocytic paraganglioma is a rare and benign gastrointestinal tumor that usually arises in the second portion of the duodenum. Histogenesis of this tumor is yet unclear. It is incidentally found during radiographic examinations or during endoscopy looking for the cause of gastrointestinal hemorrhage, which usually manifests as mucosal ulcerations. To our knowledge, there was only one case of duodenal gangliocytic paraganglioma presenting with melena in Korea. We experienced a case of gangliocytic paraganglioma arising from the ampulla of Vater, presenting as obstructive jaundice, which was subsequently removed surgically. Thus, we report this case with a review of literatures. (Korean J Gastrointest Endosc 2005;30:4751)