Nongastric mucosa-associated lymphoid tissue (MALT)-derived lymphomas arise from various extranodal locations and are usually related to a particular pathogenesis with a possible external (environmental or autoimmune) event inducing the disease. We reviewed 165 patients with nongastric MALT lymphoma among the 243 patients with MALT lymphoma in our database and reviewed reports in the literature to analyze the clinical features of nongastric MALT lymphomas. The site of clinical presentation was related to the lymphoma location and was usually indolent. Dissemination of the disease at diagnosis was noticed in 48% of cases because of the involvement of multiple mucosal sites (48%) or because of a nonmucosal site involvement such as bone marrow, spleen, or liver (52%). With a median follow-up of 4 years, the estimated 5-year overall survival and 5-year freedom-from-progression rates were 89% and 50%, respectively, without any difference between patients with localized or disseminated disease or among different locations. Treatment recommendations for localized disease are based on surgery, local therapy, or chlorambucil. For disseminated disease, treatment recommendations include chemotherapy with fludarabine or chlorambucil or chemotherapy with CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) in cases of large tumor mass