This case study of a 14-yr-old boy evaluates the clinical effectiveness of a psychological treatment within a behaviour medicine approach to epilepsy of a subject with progressive myoclonic epilepsy. The pattern of seizure behaviour was identified in the behaviour analysis. Self-management skills were taught to the client and in vivo exposure was subsequently used in a desensitization process. The main findings in this study were that the debilitating effects of the myoclonic jerks can be reduced and functioning level improved when fear of seizures is reduced. These results indicate that psychological treatment can increase the level of functioning in clients with progressive myoclonic epilepsy.