OBJECTIVE Primary central nervous system lymphoma is a rare non-hodgkin lymphoma.In recent years,the incidence has been increasing,and the growth rate is the highest in intracranial tumors.The clinical features of the disease are non-specific,with short duration,quick progress,short survival and poor prognosis.The aim of this study is by summarizing the treatment and research progress of primary central nervous system lymphoma to improve the understanding.METHODS "primary central nervous system lymphoma,PCNSL,treatment,chemotherapy,radiotherapy,targeted therapy,autologous stem cell transplantation" were searched as key words by Pubmed and CNKI series full-text database retrieval system from 1992 to 2017.The inclusion criteria:the treatment of primary central nervous system lymphoma.Exclusion criteria:literature review; lack of research data.According to the criteria,totally 45 papers were analyzed,including 39 English papers and 6 Chinese papers.RESULTS There is no optimal treatment for the primary central nervous system lymphoma.Chemotherapy based on high-dose methotrexate has become the first-line therapy of PCNSL.Chemotherapy combined with radiotherapy can prolong overall survival,but simultaneously result in higher incidence of neurotoxicity.High-dose chemotherapy followed by autologous stem cell transplantation can be used in young patients or refractory and recurrent PCNSL.The application of such as rituximab,temozolomide,thiotepa,pemetrexed can prolong the survival.The new target drugs need further study.CONCLUSION The treatment of primary central nervous system lymphoma is still based on methotrexate combined chemotherapy,and the targeted drug therapy is expected. © 2018, Editorial Board of Chinese Journal of Cancer Prevention and Treatment. All right reserved.