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Ankara Numune Eğitim ve Araştırma Hastanesi, Beyin ve Sinir Cerrahisi Kliniği, Ankara, Türkiye Primary central nervous system lymphoma (PCNSL) is defined as the extra nodal non-Hodgkin lymphoma, which is confined to brain, orbit, spinal cord and cerebrospinal fluid without evidence of a primary focus elsewhere in the body. PCNSL occurs 2%–6% of all primary brain tumors and 1%–2% of all non-Hodgkin lymphomas. The clinical presentation of patients with PCNSL varies depending on the location and size of the tumor, as well as the immunological status of the patient. The diagnosis can made with imaging (computerized tomography or magnetic resonance imaging), analyses of cerebrospinal fluid and histopathologic investigation. Histology of the PCNSL almost consists of intermediate to highgrade extra nodal non-Hodgkin's lymphoma of B-cell origin. Treatment options include corticosteroid, radiotherapy and chemotherapy. In our study we reported a 35 years old male patient with no evidence of lymphoma outside the CNS and systemic acquired immunodeficiency syndrome. Anahtar Kelimeler : Central nervous system, Lymphoma, Immunosupression