Some children with malignancies initially present with neurologic signs. Cavernous sinus syndrome is a rare manifestation of lymphomas, more commonly reported in adults. A patient presenting with third and fourth cranial nerve palsies was initially thought to manifest Tolosa-Hunt syndrome, but during follow-up a diagnosis of lymphoma without systemic involvement was established. This patient is the youngest, to our knowledge, to be diagnosed with primary cavernous sinus lymphoma. He remains in remission 5 years after chemotherapy. Malignancies (especially non-Hodgkin's lymphoma) should be considered in young children with cavernous sinus syndrome, even without systemic involvement. (C) 2010 by Elsevier Inc. All rights reserved.