Primary effusion lymphoma (PEL) is an aggressive neoplasm with a high rate of fatality. PEL cells are known to have morphological diversities, which range from immunoblastic or plasmablastic to anaplastic. Most of these cases are described in immunocompromised as well as immunocompetent patients. Plasmablastic lymphoma remains a diagnostic challenge, especially when encountered with the presentation as PEL. In spite of therapeutic advances, PEL remains an aggressive disease with a high rate of fatality. We describe one case of this extremely rare neoplasm in an immunocompromised patient presenting in the form of primary central nervous system effusion plasmablastic lymphoma. To the best of our knowledge, this is the first case ever been reported in the literature
