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Sturge-Weber syndrome is a sporadic neurocutaneous syndrome characterized by facial capillary malformation, ocular vascular malformation and leptomeningeal angioma. The involvement of whole ophthalmic branch of trigeminal nerve and bilateral face by port-wine stains often strongly predicts the presence of underlying neurological and/or ocular disorders that require on-going ophthalmological surveillance and/or neurological management. Magnetic resonance imaging ��MRI��, especially after contrast injection, is the preferred and most sensitive imaging technique for the diagnosis of Sturge-Weber syndrome. Facial port wine stain can be treated with pulsed dye laser, and the control of epilepsy and glaucoma may be achieved by medical treatment and surgical operation.