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Primary central nervous system lymphoma (PCNSL) is a rare, extranodal non- Hodgkin's lymphoma confined to craniospinal axis. PCNSL accounts for less than 5% of all primary CNS malignancies and 1 to 2% of all lymphomas. Amongst the causes of major cognitive dysfunction, primary brain tumor exists as a less frequent cause. We report a case of PCNSL as a rare and unusual presentation of rapidly progressive cognitive dysfunction which revealed clinicopathoradiological correlation. The patient revealed clinicoradiologically evident marked i