Sturge-Weber Syndrome (SWS) is a rare, non-hereditary, congenital neuro cutaneous disorder characterized by vascular hamartomatous proliferations affecting classically, the leptomeninges and the skin of the face and may be associated with glaucoma, seizures and mental retardation. Intraoral findings are variable. A case of Roach type II Sturge Weber syndrome is reported here that did not show any neurological disorder but revealed a prominent angiomatous enlargement of the ipsilateral maxillary gingiva. SWS is a condition where dental management and surgical procedures of the patient can be risky, thus a sound knowledge of the disease and its management protocols better equips the clinician to avoid serious complications.
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