Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (EMZL/MALT-type) occurs in a wide variety of body sites; it is well recognized as a form of primary lung lymphoma. However, until recently, pleural presentation of this form of low-grade lymphoma has not been documented. A small series of case reports has brought to attention the potential for primary occurrence or initial presentation in the pleura of EMZL/MALT-type. In this report, we describe an additional patient with EMZL/MALT-type with initial pleural presentation and review the literature. Clinicians and pathologists dealing with lymphoproliferative disorders involving the pleura should be aware of this rare entity.