As a rare extranodal non-Hodgkin’s lymphoma， primary central nervous system lymphoma （PCNSL） is highly invasive and can involve the brain， eyes， leptomeninges， and spinal cord. PCNSL has a high response rate to chemoradiotherapy and immunotherapy， but short-term recurrence is often observed in patients receiving a single regimen. Currently accepted treatment methods include induction therapy and consolidation therapy. This article reviews the research advances in the pathogenesis， progression， and treatment of PCNSL.