Abstract Sturge–Weber syndrome (SWS) or encephalotrigeminal angiomatosis is a rare congenital disorder that affects the skin, brain, and eye. It is characterized by facial port‐wine stains, leptomeningeal angiomas, and glaucoma. SWS can also cause neurological complications, such as seizures, headaches, cognitive impairment, and stroke. Antiepileptic drugs that are used to treat seizures associated with SWS lead to the development of gingival hyperplasia which requires non‐surgical and/or surgical dental treatment. The purpose of this case report and narrative review is to provide an overview of the etiology, pathophysiology, clinical features, diagnosis, and treatment of SWS. This paper will also discuss the oral manifestations and dental considerations of SWS.
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