Multiple odontogenic keratocysts in mental retardation-overgrowth (Simpson-Golabi-Behmel) syndrome

We report on a 10-year-old boy with mental retardation-overgrowth (Simpson-Golabi-Behmel) syndrome. The child had the typical clinical features including, postnatal overgrowth, mental retardation, and a characteristic facial appearance. He was admitted for treatment of multiple mandibular and maxillary cysts. Histopathological examination of the cyst tissue showed keratinized epithelium. Odontogenic keratocysts may have to be added to the typical features of this syndrome.