Central nervous system involvement in chronic lymphocytic leukemia is rare. We describe a patient with early stage chronic lymphocytic leukemia who developed visual loss and transient paresthesias. Lumbar puncture with cytology and flow cytometry documented leukemic meningitis, which responded to standard treatment; including cranial irradiation and intrathecal chemotherapy. We review the literature of all previously reported cases of brain or meningeal involvement by chronic lymphocytic leukemia and suggest that this complication may frequently go unrecognized.