The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) or Gorlin-Goltz syndrome, principally characterized by basal cell carcinomas, multiple jaw cysts and skeletal anomalies, is an interesting pathology for the dentist who is often the first clinician involved in the diagnosis of this syndrome. Because of the multisystem involvement and variable expressivity of NBCCS, patients affected by this syndrome must be evaluated by many medical and dental specialists in order to properly sequence their treatment.