Plasmablastic lymphoma was initially described in 1997 associated with infection with HIV. This lymphoma usually presents within the oral cavity of HIV-infected individuals and carries a poor prognosis with current chemotherapeutic approaches. Given its rarity, with just over 200 hundred patients reported in the literature to date, prospective studies are unlikely to be undertaken. With the emergence of this lymphoma, more cases are being identified in HIV-negative patients. The present publication reviews the current knowledge on the pathogenesis, clinicopathological characteristics, prognostic factors and management of plasmablastic lymphoma.