Gorlin-Goltz syndrome or basal cell nevi syndrome is an ectodermal neoplasia of autosomal dominant inheritance. It is a multisystem disease with a prevalence of 1 case per 57 000 to 150 000 people. It predominates in patients between the second and third decade of life, with a predilection for the male sex., Iit’s characterized by the presence of multiple basal cell nevus carcinomas, development of odontogenic keratocystic and palmo-plantar pits or dimples and calcification of the falx brain. Odontogenic keratocysts (OKC) occur in more than 80% of cases of patients with Gorlin-Goltz syndrome (GGS). OKC can present as the first sign of GGS. Approximately 65% of cases affect the mandible, with high frequency in the molar and ramus region. A clinical case is presented of a 35-year-old male patient with a current condition of missing teeth. Physical examination revealed syndromic facies. O...