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Lymphomatous meningitis (LM) is a common problem in neuro-oncology, occurring in approximately 5% of all patients with systemic lymphoma. Notwithstanding frequent focal signs and symptoms in LM, LM is a disease affecting the entire neuraxis and therefore staging and treatment need to encompass all cerebrospinal fluid (CSF) compartments. CNS staging of LM includes contrast enhanced cranial computerized tomography (CE-CT) or magnetic resonance imaging (MR-Gd), contrast enhanced spine magnetic resonance imaging (MRS) or computerized tomographic myelography (CT-M) and radionuclide CSF flow study. Treatment of LM includes involved-field radiotherapy of bulky or symptomatic disease sites and intrathecal chemotherapy (intra-CSF) drug therapy. The inclusion of concomitant systemic therapy (high dose methotrexate or cytarabine) may benefit patients with LM and may obviate the need for intra-CSF chemotherapy. At present, intra-CSF drug therapy is confined to three chemotherapy agents (i.e. methotrexate, cytarabine and thiotepa) administered by a variety of schedules either by intralumbar or intraventricular drug delivery. Although treatment of LM is palliative, with an expected median patient survival of 6–10 months, it often affords stabilization and protection from further neurologic deterioration.