Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome is inherited in an autosomal dominant way, although sporadic cases have been reported. This syndrome shows a high penetrance and variable expressivity and is a multisystemic process characterized by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and plantar pits and calcification of the falx cerebri. Together with these major features a great number of processes considered as minor features have also been described, such as numerous skeletal, dermatology related and neurological anomalies. Due to the importance of oral maxillofacial manifestations of this syndrome it is fundamental to know its characteristics in order to make a diagnosis and initiate an early preventive treatment. This paper reports the case of a 20-year-old male patient with Gorlin-Goltz syndrome, emphasizing the clinical and radiographic manifestations. This case report highlights the importance of dental health professionals in the early diagnosis of Gorlin-Goltz syndrome and in a preventive multidisciplinary approach to provide a better prognosis for the patient.