We report the clinical and imaging features in 16 years old girl presented with seizures, right-sided hemiparesis, hemi atrophy of the right side of the body and mental retardation. Brain MRI showed loss of left side cerebral volume with ipsilateral hypertrophy and hyperpneumatization of the paranasal sinuses and mastoid cells, suggestive of congenital type of cerebral hemi atrophy (CH) diagnosis of DDMS was made.