Extranodal lymphomas arising from mucosa associated lymphoid tissue (MALT) have become a focus of interest in recent years due to their unique pathological and clinical properties. The link between Helicobacter pylori and the development of gastric MALT-type lymphoma has revolutionized treatment options as up to 80% of patients with early gastric MALT-type lymphoma achieve complete remission of the tumor following eradication of H. pylori. As opposed to surgical intervention, which has been the preferred form of treatment in the past, organ conserving approaches are increasingly being applied, as both irradiation and chemotherapy have given excellent results. However, mature data from prospective, randomized studies taking into account the concept of MALT lymphoma as a distinct entity are still lacking in order to define the optimal approach to the management of MALT-type lymphoma.