Most primary gastric mucosa-associated lymphoid tissue(MALT)lymphomas are associated with a chronic Helicobacter pylori(H. pylori)infection. The eradication of H. pylori is the first-line treatment for H. pylori-positive cases with early-stage disease. In addition, successful treatment of H. pylori-negative early stage MALT lymphomas by eradication has been reported in several small cases series. However, the association of primary gastrointestinal MALT lymphomas with H. pylori in areas other than the stomach is not clear, and the efficacy of eradication therapy for these patients has not been established. We performed H. pylori eradication therapy for H. pylori-negative cecum MALT lymphoma. Three months later, a histopathological examination showed no evidence of MALT lymphoma, and the patient was classified as being in remission. So far, the patient has been in remission for 1 year and 6 months. Our case is the first report of successfully treating H. pylori- negative cecum MALT lymphoma with eradication therapy.