IntroductionSturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a rare congenital disorder characterized by abnormal vasculature in the brain (leptomeningeal), skin & eye (1). Classically facial capillary malformation also known as port wine stain, is seen in the ophthalmic and maxillary division of the trigeminal nerve. (2) The most common presenting neurological manifestation is seizure with the age of onset ranging from birth to late adulthood. It is third most neurocutaneous syndrome after neurofibromatosis and tuberous sclerosis. (3) (4)Other clinical features include Port-Wine stain , glaucoma, visual impairment, migraine, hemiparesis, and cognitive impairments. (5) Schirmer provided the first detailed description of SWS in 1860, Sturge further described SWS- related dermatological, ophthalmic and neurological manifestations in 1879 and Weber reported radiological altercations seen in these patients in 1929.Although the incidence is not reported accurately, it is estimated to be 1 in 20,000-50,000 live birth. (6)
