Sturge Weber Syndrome also called as encephalotrigeminal angiomatosis is a sporadically occurring neurocutaneous syndrome, characterized by vascular malformation with capillary venous angiomas that involve face, choroid of eye and leptomeninges with resulting neurological and orbital manifestations. We hereby report a young unmarried girl who diagnosed as a case of SWS on the basis of Port wine stain since birth, past history of seizures since the age of four years and at this time presented with status epilepticus, mental retardation (I.Q.--30 to 35), EEG abnormality and characteristic imaging findings. This may be a common condition for paediatrician and neurologist but as a physician we usually do not see it that often.