Abstract:Sturge-Weber syndrome is characterized by unilateral facial port wine stain, ipsilateral leptomeningeal vascular anomalies, ipsilateral choroidal angiomas which lead to glaucoma and one or more symptoms (epilepsy, hemiparesis, hemiplegia or mental retardation). We hereby, present a case of 5 year old girl with seizure disorder, right sided hemiparesis along with angiomas on both sides of her face, more extensive and prominent on her upper and lower limbs. Ocular examination revealed raised intraocular pressure and choroidal hemangiomas in both eyes. Non contrast enhanced computerized tomography of head showed left sided cortical hemi-atrophy with mild atrophy of the right occipito-parietal cortex. Gyriform calcifications were seen in bilateral temporo-occipitoparietal regions. The case was diagnosed as bilateral Sturge-Weber syndrome with extensive extrafacial distribution of cutaneous lesions.