A 14-year-old adolescent was seen with an 8-month history of almost daily incapacitating headaches due to idiopathic intracranial hypertension in Behçet syndrome. All his clinical signs and symptoms, including headache, resolved 2 to 4 weeks after topiramate was initiated. An effect on carbonic anhydrase isoenzymes II and IV, reducing cerebrospinal fluid production, could potentially explain the beneficial effect of topiramate in intracranial hypertension. Further studies are necessary, however, to confirm the significance of topiramate in this indication.