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As part of the non\u2010Hodgkin B cell lymphoma group, it is much more indolent in presentation and progression than its aggressive cousin diffuse B\u2010cell lymphoma (DBCL) of the CNS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This systematic review collates all the published outcome and treatment data on CNS MALT to shed more light on this rare entity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>With 3\u2010year overall survival rates of over 94%, females represent two thirds of cases. Dural involvement is much more common and carries a positive prognostic advantage, as does younger age. Treatment strategies still appear to be widely varied, but surgery and adjuvant therapy appear to provide the longest lasting progression\u2010free survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The rare entity of CNS MALT lymphoma can present in a range of ways. While this means a variety of symptoms, its general prognostic course is usually more indolent than other B cell lymphomas. It frequently affects a younger and more female predominant population, yet no demographic, presentation, or histological characteristics are associated with poorer prognosis. 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