Thirty-five of 77 patients (46%) with Burkitt’s lymphoma presented or developed evidence of central nervous system involvement by tumor. Neurologic abnormalities included paraplegia, cranial neuropathy, altered levels of consciousness and malignant pleocytosis. An analysis of this series disclosed the following: Paraplegia is a common presenting feature of Burkitt’s lymphoma and is responsive to systemic chemotherapy. The association of cranial neuropathy and malignant pleocytosis with facial tumors points to direct tumor extension to intracranial structures (duraarachnoid) as the pathogenesis of these lesions. Intrathecal chemotherapy temporarily reverses malignant pleocytosis but systemic chemotherapy is required to treat cranial neuropathy. A poor prognosis follows presentation or development of malignant pleocytosis. The limitations of the current forms of therapy for CNS involvement are discussed.