Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is the paradigm of lymphomas developing in extranodal areas after antigen stimulation. In the stomach, Helicobacter pylori colonization induces the appearance of MALT and, eventually, MALT-derived lymphoma. This type of lymphoma is initially a localized form of disease, but may disseminate and transform into high-grade lymphoma, making full staging (as for nodal lymphomas) and endoscopic ultrasonography to evaluate the penetration of the lymphoma through the gastric wall mandatory. In localized gastric MALT lymphoma, the first step in treatment is eradication of H. pylori, which results in 60-90% regression. This response is maintained for years in most patients, with only 10-15% relapse, frequently precipitated by H. pylori reinfection. A component of high-grade lymphoma, penetration to gastric serosa or beyond and translocation t(11;18) are the main factors that make lymphoma resistant to eradication. Surgery or radiotherapy can cure localized lymphomas in 75-90% of patients. Chemotherapy with alkylating agents, combination chemotherapy and purine analogs, and anti-CD20 antibodies can also induce remission of localized lymphomas refractory to eradication, as well as locally advanced and disseminated lymphomas. The optimum chemotherapy treatment for advanced disease has not yet been established; however, combination therapy, including purine analogs with or without anti-CD20, may be a promising option. Despite histological responses and prolonged remissions, residual molecular disease can be demonstrated in most cases treated with H. pylori eradication, radiotherapy or alkylating agents, and even after more intense chemotherapy, although this does not seem to lead to late relapses. High-grade gastric MALT lymphoma should be treated with chemotherapy, with cyclophosphamide, doxorubicin, oncovin and prednisone being the best first-line option. All gastric MALT lymphomas associated with H. pylori should receive eradication treatment in addition to other required treatment.