Primary central nervous system lymphoma (PCNSL) is a unique subtype of non-Hodgkin's lymphoma (NHL). Although PCNSL is both chemosensitive and radiosensitive, it is very difficult to treat and outcomes are substantially inferior when compared with similar extranodal NHL. Furthermore, as a subtype of brain tumors and NHL, the overall incidence has been increasing over the past several decades. This review summarizes the salient clinical features and approach to management of the immunocompetent patient with PCNSL.