Introduction: Development of gastric neuroendocrine neoplasms in subjects infected with Helicobacter pylori is rare and it occurs through pathogenetic mechanisms related to gastrin. Presentation of case: We report a case of gastric neuroendocrine tumor in a patient infected with Helicobacter pylori and normal gastrin levels. He was treated by endoscopic mucosal dissection after eradication of Helicobacter pylori infection. Histologically the tumor was consistent with a grade 2 well differentiated neuroendocrine tumor. It was characterized by the presence of lymphoid aggregates around and inside the neoplasia. Discussion: Helicobacter pylori -associated chronic gastritis can rarely cause the development of gastric neuroendocrine tumors through mechanisms unrelated to gastrin. Conclusion: The one related to a chronic Helicobacter pylori infection may be considered a distinct type of gastric neuroendocrine tumor. Highlights: