A 63 years old male patient with systemic extranodal marginal zone lymphoma, most probably originating from mucosa associated lymphoid tissue (MALT) involving central nervous system (CNS) is presented. The usual site of origin of this type of lymphoma was not identified. The diagnosis was stated after neurosurgery according to histopathology, immunohistochemistry and additional haematologic examination. Postoperative therapy included local irradiation (30Gy) of rest tumor, combined by Rituximab-CHOP (R-CHOP) protocol, which resulted in complete remission lasting three years up to now.

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