Epilepsy may present as a symptom of many neurological disorders and often an etiological factor cannot be identified. There is some evidence that immune mechanisms might play a role in the pathogenesis of certain epileptic syndromes. The evidence in support of this idea includes apparent association of seizures with certain autoimmune diseases, numerous reports of the detection of theoretically relevant serum autoantibodies, an acute or subacute onset of the seizures, a rapidly progressive course, and a favorable response to immunotherapy. In this article, we summarize (i) epilepsies where clinical and biologic data strongly support the pathogenic role of autoantibodies. (ii) epilepsies where immune-mediated inflammation occurs, but the full pathogenic cascade is either not clear or only strongly hypothesized and (iii) autoimmune diseases associated with seizures or epilepsy.