Hemimegalencephaly is a rare congenital anomaly characterized by a intractable seizure an infantile death. This disease is a migrational disorder with enlargement of unilateral hemisphere, abnormal gyration of the cortex, the presence of large and atypical neuron, hetero trophic neuron at subcortical area and proliferation of astrocyte ect. We observed a neonate with neonatal seizure, and the radiological evaluation revealed abnormal enlargement of right hemisphere, intraventricular hemorrhage of both ventricle and multifocal intraparenchymal hemorrhage on deep white matter of periventricular area on both sides. We used the phenobarbital for treatment of seizure. After phenobarbital treatment. The seizure was controlled. A review of literature was also presented briefly