The Sturge-Weber syndrome (SWS) or Encephalotrigeminal Angiomatosis is specifically congenital, non-hereditary, rare condition of unknown etiology. The classic pathognomonic features of disease include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus after one division of trigeminal nerve and epileptic convulsions.
The most characteristic oral manifestation is represented by gingival hemangiomatous lesion usually restricted to ipsilateral maxilla or mandible. A classic case of Sturge-Weber syndrome is reported here.
