AIM: To investigate treatment outcome of Helicobacter pylori ( H. Pylori)-negative low-grade gastric mucosaassociated lymphoid tissue (MALT) lymphoma. METHODS: In this study, we retrospectively reviewed the clinical outcome and clinicopathologic factors of stage ⅠE H. Pylori-negative low-grade gastric MALT lymphoma cases from August 1998 to June 2009. RESULTS: A total of eleven patients with H. Pylori-negative low-grade gastric MALT lymphoma were enrolled in the study and received anti- H. Pylori eradication treatment and/or radiotherapy or excisional therapy. Complete remission (CR) of gastric MALT lymphoma was achieved in all patients. The time to CR was 1-66 mo (median, 1 mo). CONCLUSION: Eradication therapy may be offered as an initial treatment option even in cases of localized H. Pylori-negative gastric MALT lymphoma.