Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant trait, with complete penetrance and variable expressivity, though sporadic cases have been described. This article includes a case report and an extensive review of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, keratocystic odontogenic tumor and treatment modalities.

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The prevalence of MIH in the endemic fluorosis district was found to be 7.3 %. A gradual increase in MIH was seen with age due to post enamel breakdown. Caries involvement was also seen in more than 50 % of the MIH-involved teeth. This prevalence study for molar incisor hypomineralisation (MIH) is rare in the literature, especially in a fluorosis endemic district in Tamilnadu.

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