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Ann Saudi Med 28(2) March-April 2008 www.kfshrc.edu.sa/annals 150 Sturge-Weber syndrome or encephalofacial angiomatosis syndrome is a congenital and progressive disorder characterized by a port-wine stain in the area innervated by the first sensory branch of the trigeminal nerve, generalized or contralateral focal seizures as well as hemiplegia, homonymous hemianopsia, ipsilateral intracerebral calcifications, and mental retardation.1 Even though familial cases have been reported, it has not been proved that it is hereditary. Although clasically, Sturge-Weber syndrome consist of unilateral facial nevus and ipsilateral intracerebral calcifications radiologically, atypical variants have been reported.2-4

Diagnosis: An atypical case of Sturge-Weber syndrome with bilateral facial hemangioma, bilateral intracranial calcification, and megalocornea