Primary central nervous system lymphoma（PCNSL）is a rare and and aggressive extranodal non-Hodgkin lymphoma（NHL），which is usually confined to the brain，eyes，spinal cord or leptomeninges without systemic involvement. The diagnosis，treatment and prognosis of PCNSL are different from other types of NHL. Traditional treatment methods，such as surgery，whole brain radiotherapy，chemotherapy based on high dose methotrexate and rituximab，can leads to high initial response rate，but the duration of response is short，the recurrence rate is high，the neurotoxicity are large，and the overall survival period is poor. In addition，the effect of traditional treatment for recurrent/refractory PCNSL patients is not satisfactory，so it is urgent to find a more effective and longer lasting treatment. At present，with the development of the research on the molecular mechanism and the immunotherapies，novel treatments，including Burton tyrosine kinase（BTK）inhibitors，programmed cell death protein 1（PD-1）inhibitors and immunomodulators，bring new light to PCNSL patients.