Primary central nervous system lymphoma (PCNSL) is an invasive extranodal non-Hodgkin lymphoma (NHL) confined to the central nervous system (CNS); it arises from the brain parenchyma, cranial nerves, leptomeninges, eyes, or spinal cord without involvement of other sites (Ferreri and Illerhaus, 2016). PCNSL accounts for approximately 4% to 6% of all cases of NHL, and has an incidence rate of 0.4 to 0.5 per 100000 (Mendez et al., 2018). Pathologically, it is usually characterized by highly proliferative tumor cells with a vasocentric growth pattern and diffuse infiltration into adjacent CNS tissues (Louis et al., 2016). In more than 90% of cases, the tumors are pathologically diffuse large B-cell lymphoma (DLBCL) (Alizadeh et al., 2000); however, the treatment of PCNSL differs from that of DLBCL, and the prognosis is poorer. The presence of the blood-brain barrier (BBB) poses a major hindrance to the treatment of PCNSL, because it limits the entry of macromolecular antineoplastic drugs and their access to the CNS (Schmitz, 2015). At present, the treatment of PCNSL is mainly divided into remission induction, consolidation, and maintenance stages. Owing to the shift in treatment approaches from surgical resection and radiotherapy to combined high-dose methotrexate (HD-MTX)-based chemotherapy, the remission rate of PCNSL has significantly increased. The 5-year overall survival (OS) of patients has increased from 19.1% in the 1990s to 30.1% in the 2000s (Shiels et al., 2016). However, almost 50% of patients experience relapse after treatment (Ambady et al., 2017); these recurrent cases have a poor prognosis. The administration of effective consolidation treatment and the need for maintenance therapy are therefore issues that need to be addressed urgently. This article reviews the diagnosis and treatment of PCNSL, with focus on the progress of research pertaining to consolidation treatment.