Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome consisting of port wine stain (facial nevus flammeus), congenital glaucoma and underlying anomalous leptomeningeal venous plexus with lack of normal cortical venous drainage. Neuromuscular, ocular, dermatologic and oral manifestations are variously present with this syndrome. The syndrome presents with various oral findings and the diagnosis of these cases is based primarily on clinical findings. Considering the complications that may be encountered during the treatment and the patient's own perception of the condition, it is imperative that the diagnostician and surgeon have thorough knowledge of the condition. Here we report a case of SWS with facial angiomas, seizures and intracranial calcifications.