Pervasive developmental disorder is characterized by various symptoms that often include self-injurious behavior (SIB). Episodes of SIB occur in the context of high emotional arousal, anger, or fear and may be related to epilepsy. We report the case of a 20-year-old man with pervasive developmental disorder presenting with SIB non-responsive to antipsychotic medication. Positron emission tomography showed a right temporoparietal hypometabolic focal lesion suggestive of an epileptic focus. Two weeks after initiation of levetiracetam (Keppra®), SIB disappeared, without recurrence 24 months later. Levetiracetam (Keppra®) may be beneficial for such patients.