Ocular lymphoma is an uncommon clinical entity with a propensity for intracranial extension. Palliation has been reported following radiotherapy, but the ultimate prognosis is poor, and significant treatment-related morbidity is common. Recent pharmacokinetic studies have suggested that sustained therapeutic drug concentrations are achievable in cerebrospinal fluid after systemic administration of high-dose cytosine arabinoside (Ara-C). These data led the authors to attempt treatment of a case of recurrent ocular lymphoma with high-dose Ara-C. Therapeutic drug levels were documented in intraocular fluids, and prolonged objective regression of tumor was seen. Systemic high-dose Ara-C deserves consideration for the treatment of ocular lymphoma.