Sturge-Weber syndrome also called as encephalotrigeminal angiomatosis is a rare congenital neurological and skin disorder caused by persistence of transitory primordial arteriovenous connection of the fetal intracranial vasculature. It is characterized by vascular malformation with capillary venous angiomas that involve face, choroid of eye and leptomeninges with resulting neurological and orbital manifestations. The diagnosis is usually considered in a child presenting with seizures and facial capillary malformation along the trigeminal nerve distribution. The characteristic imaging features on computed tomography and magnetic resonance imaging lead to the diagnosis. We report a case presenting the atypical and radiographic features of this syndrome.