Background: Sturge-Weber syndrome is one of the rare neurocutaneous disorders with frequency of approximately 1 per 50,000. Sturge-Weber syndrome consists of a constellation of symptoms and signs including a facial nevus (port wine stain), seizure and hemiparesis. In many cases it may associate with mental retardation. Case presentation: A 7- year old girl with mental retardation and a large facial nevus at the birth time, recurrent severe seizures and drolling in 9 months of life without any treatment, was admitted because of severity of seizure, headache and weakness. Conducted follow up on here, indicated that she had Sturge –Weber syndrome with oro- maxilla -facial osteodystrophy. Conclusion: One of the rare radiological symptoms in Sturge Weber syndrome is oro-maxillo-facial osteodystrophy, by this time only few cases had been reported. All of the symptoms of this syndrome were found in our described patient

Keywords: Syndrome, Sturge -Weber, seizure, osteodystrophy.

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