Dyke-Davidoff syndorme is a rare congenital disorder which developed hemiatropy of brain, enlargement of ventricles and prominent pneumatization of petrous pyramid of the temporal bone. It mainfests unilateral invountary movement such as seizure, athetosis, chorea and mental retardation. This disorder have strong evidence of significance to differential diagnosis of cerebral palsy because of different therapeutic approach and outcome. Recently we examined 10 years old girl who developed seizure at 2 years old and gradually progressed with hemiparesis, hypoplaisa of right side of the body. The brain CT scan showed marked dilatation of lateral ventricle, prominent sylvian fissure and cortical sulci with somewhat thickened skull vault and pneumatization of petrous pyramid of the temporal bone on left side. EEG showed high voltage slow wave and spikes. So we report a case of Dyke-Davidoff syndrome with review of literature.