Recent progress in the research regarding the molecular pathogenesis and management of gastric mucosaassociated lymphoid tissue(MALT)lymphoma is reviewed.In approximately 90%of cases,Helicobacter pylori(H.pylori)infection plays the causative role in the pathogenesis,and H.pylori eradication is nowadays the first-line treatment for this disease,which leads to complete disease remission in 50%-90%of cases.In H.pylori-dependent cases,microbe-generated immune responses,including interaction between B and T cells involving CD40 and CD40L co-stimulatory molecules,are considered to induce the development of MALT lymphoma.In H.pylori-independent cases,activation of the nuclear factor-κB pathway by oncogenic products of specific chromosomal translocations such as t(11;18)/API2-MALT1,or inactivation of tumor necrosis factor alpha-induced protein 3(A20)are considered to contribute to the lymphomagenesis.Recently,a largescale Japanese multicenter study confirmed that the long-term clinical outcome of gastric MALT lymphoma after H.pylori eradication is excellent.Treatment modalities for patients not responding to H.pylori eradication include a“watch and wait”strategy,radiotherapy,chemotherapy,rituximab immunotherapy,and a combination of these.Because of the indolent behavior of MALT lymphoma,second-line treatment should be tailored in consideration of the clinical stage and extent of the disease in each patient. Recent progress in the research regarding the molecular pathogenesis and management of gastric mucosaassociated lymphoid tissue (MALT) lymphoma is reviewed. Approximately 90% of cases, Helicobacter pylori (H. pylori) infection plays the causative role in the pathogenesis, and H. pylori eradication is nowadays the first-line treatment for this disease, which leads to complete disease remission in 50% -90% of cases.In H. pylori-dependent cases, microbe-generated immune responses, including interaction between B and T cells involving CD40 and CD40L co-stimulatory molecules, are considered to induce the development of MALT lymphoma. In H. pylori-independent cases, activation of the nuclear factor-κB pathway by oncogenic products of specific chromosomal translocations such as t (11; 18) / API2 -MALT1, or inactivation of tumor necrosis factor alpha-induced protein 3 (A20) were considered to contribute to the lymphomagenesis. Published, a large scale Japanese multicenter study confirmed that the long-term clinical outcom e of gastric MALT lymphoma after H. pylori eradication is excellent. Treatment modalities for patients not responding to H. pylori eradication include a “watch and wait ” strategy, radiotherapy, chemotherapy, rituximab immunotherapy, and a combination of these. the indolent behavior of MALT lymphoma, second-line treatment should be tailored in consideration of the clinical stage and extent of the disease in each patient.