Abstract
Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant inherited disorder characterized by the presence of multiple keratocystic odontogenic tumors (KCOT) in the jaws, multiple basal cell nevi carcinomas, and skeletal abnormalities. Early diagnosis of Gorlin-Goltz syndrome is essential as it may progress to aggressive basal cell carcinomas and neoplasias. Gorlin-Goltz syndrome has rarely been reported in Saudi Arabia. This article reports a case of a 13-year-old Saudi female patient with Gorlin-Goltz syndrome and includes an extensive literature review of the syndrome. To the extent of our knowledge, this is the first case reported by dentists in the Kingdom of Saudi Arabia.