Familial cortical myoclonic tremor with epilepsy (FCMTE) is a rare disorder characterized by irregular postural tremor of the limbs, family history of seizures, autosomal dominant inheritance, and a rather benign course. A 23 year-old man who had a history of seizure attack since age 16 showed postural and kinetic tremor and mental retardation (MR). His older sister as well as his mother had similar clinical feature. We report the first case of FCMTE in Korea.