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Purpose of Review: Primary central nervous system (CNS) lymphoma is a rare and aggressive extranodal non-Hodgkin lymphoma confined to the brain, eyes, spinal cord, or leptomeninges without systemic involvement. This article provides an overview of the clinical features, diagnosis, and management of primary CNS lymphoma in patients who are immunocompetent, focusing on recent advances in treatment.Recent Findings: Primary CNS lymphoma is sensitive to radiation therapy; however, whole-brain radiation therapy inadequately controls the disease when used alone and causes delayed neurotoxicity with significant neurocognitive impairment, especially in patients who are elderly. A number of clinical trials have demonstrated durable disease control and less neurotoxicity with methotrexate-based induction chemotherapy with or without autologous stem cell transplantation or reduced-dose whole-brain radiation therapy.Summary: Prompt diagnosis and initiation of treatment are vital to improving clinical outcomes in patients with primary CNS lymphoma. The optimal treatment has yet to be defined, but high-dose methotrexate-based induction chemotherapy is considered standard for newly diagnosed primary CNS lymphoma. Ongoing randomized trials will attempt to address the roles of rituximab and consolidative treatment using autologous stem cell transplantation or reduced-dose whole-brain radiation therapy. Despite high tumor response rates to initial treatment, many patients will relapse. The choice of salvage treatment will depend on age, previous treatment and response, performance status, and comorbidities at the time of relapse.