Behçet's disease (BD) is a multisystemic disease of unknown etiopathogenesis with various clinical features including manifestations from central nervous system involvement. We report the case of a patient presented with a 20-year history of BD and a 10-year history of epileptic seizures refractory to various antiepileptic drugs. Under systemic treatment with interferon-alpha 2a (IFN-alpha) a complete remission of the cutaneous manifestations and a seizure-free state were achieved. The impressive therapeutic response of both the seizures and the non-neurological manifestations to IFN-alpha was also observed upon re-administration of this cytokine subsequent to a severe BD relapse. In view of this response and the lack of any other obvious etiology of the seizures in our patient, it seems reasonable to consider them as being the sole manifestation of neuro-BD. The patient is presently completing a 40-month seizure-free follow-up, despite withdrawal of all antiepileptic drugs for the last 35 months. Further studies on large numbers of patients are now warranted to define the therapeutic efficacy and safety of IFN-alpha in neuro-BD and particularly in neuro-BD-related epileptic seizures.