Diagnostic Sphinx: Sturge Weber Syndrome

Sturge weber syndrome is a sporadic congenital neuro cutaneous disorders. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma (cerebral malformations and tumours). Regime dental procedures are associated with increased vulnerability of hemorrhage, making treatment in such patients an imperative task for oral health care practitioners. Unforeseen complications can be prevented by having radical knowledge of this rare syndrome. We hereby report a case of 35 year old female presented with port wine stain and diagnosed as type 2 Sturge-Weber syndrome after investigation. Keywords: Sturge Weber syndrome, Congenital, Encephalotrigeminal angiomatosis, Vascular lesions, Oral manifestations