Sturge-Weber syndrome (SWS) is a sporadically occurring neurocutaneous syndrome characterized by port-wine stain over the face, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angiomas. It is usually diagnosed in infancy, but it may occasionally present in adulthood with seizures or stroke-like episodes. Here, we report a 46-year-old male patient, having SWS coexisting with moyamoya disease, attending our hospital due to sudden loss of consciousness. We also searched PubMed (from its earliest date to August 2014) for case reports mentioning that SWS presents in adulthood. We identified 31 patients. The common clinical manifestation are seizures, stroke-like episodes, and migraine-like headaches.On the basis of our findings in this patient, we would recommend that patients with a port-wine nevus of the face should be given further investigation to rule out an intracranial vascular malformation, especially if seizures, stroke-like episodes, or migraine-like headaches are present.