Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, being due to a genetic alteration which produced by mutation in the “Patched" tumor suppressor gene, and it is inherited in an autosomal dominant way. This syndrome is about multisystemic process that is characterized by the presence of multiple pigmented basocellular carcinomas, odontogenic keratocysts in the jaws, palmar and/or plantar pits and calcification of falx cerebri. Together with these major features a great number of processes considered as minor features have also been described which include numerous skeletal, dermatological and neurological anomalies among others. Due to the importance of oral and maxillofacial manifestations of this syndrome, it is fundamental to know its characteristic in order to make a diagnosis, an early preventive treatment and establish right genetic advice.