Fahr's syndrome is a neuropsychiatric syndrome characterized by symmetrical and bilateral intracerebral calcifications located in the basal ganglia and usually associated with a phosphorus and calcium metabolism disorder. Clinical manifestations of Fahr's syndrome vary; it may start at different ages and have a variety of presentations. This article discusses rare presentation of Fahr's syndrome with epileptic seizure. These cases are important because they appear to be the first cases in the literature of Fahr's syndrome presenting with generalized tonic clonic seizure.