Primary central nervous system lymphoma is a rare lymphoproliferative disorder confined to the central nervous system. Here we report a case of a 73-year-old female with an intracerebral lymphoma, discussing clinical presentation, diagnosis and treatment. Primary central nervous system lymphoma is a subtype of non-Hodgkin lymphoma which, in more than 90% of the cases, are diffuse large B-cell lymphomas. Clinical presentation is broad; the most common presentation in an immunocompetent patient is a focal neurological deficit. The neuroimaging technique of choice for diagnosis is contrast enhanced magnetic resonance. Histopathologic diagnosis is essential, which is mostly performed by a stereotactic guided biopsy. The backbone of treatment for primary central nervous system lymphoma is high-dose methotrexate, which can be combined with other chemotherapy, whole brain radiotherapy, corticosteroids and rituximab.

(BELG J HEMATOL 2016; 7(3):118–22)