Sturge-Weber Syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a rare neurocutaneous syndrome caused by a somatic mutation in the GNAQ gene [1]. The incidence of SWS is 1:50,000, and it affects both sexes equally [2]. The characteristic hallmarks of the disease include 1) unilateral facial nevus, 2) dural and leptomeningeal angiomatosis, 3) hemangiomas of the choroid, and 4) congenital glaucoma [3,4]. However, these findings may not always be present in patients; and although often viewed as a single pathologic entity, SWS was recently subdivided to three types: Type I comprises facial and leptomeningeal angioma, Type II features facial angioma without apparent intracranial involvement, and Type III consists of neurological involvement only, without any dermatologic findings [4–6].
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