Mucosa-associated lymphoid tissue (MALT) lymphomas were first described by Isaacson and Wright in 1983 in a small series of patients with low-grade B-cell gastrointestinal lymphomas. Although MALT lymphomas occur most frequently in the stomach, they have also been described in various non-gastrointestinal sites, such as the salivary gland, conjunctiva, thyroid, orbit, lung, breast, kidney, skin, liver, uterus, and prostate. The risk of a diagnostic dilemma is reduced by the favorable prognosis of this low-grade lymphoma and its tendency to remain localized to the primary site for a long time. On therapeutic grounds, in contrast to nodal lymphomas, low-grade MALT lymphomas also respond favorably to local treatments, such as surgery and/or local radiation therapy. The outcome and prognosis of low-grade MALT lymphomas are more favorable than those for other extranodal lymphomas. The purpose of this review is to point out the state of the art regarding bronchus-associated lymphoid tissues lymphomas in terms of etiology, pathogenesis, clinics, and therapeutics, including our single-center experience of 23 cases.