Gorlin-Goltz Syndrome (GGS) also known as Nevoid Basal Cell Carcinoma Syndrome is a rare autosomal dominant disorder. Is characterized for presenting some clinical features such as palmar pits, fused eyebrows, multiple odontogenic keratocyst, which are considered some of the most common signs. In our case, a rare familial case highlights awareness when a member of the family is diagnosed with this condition, and the probability to find in one the first degree relative. Once again it is confirmed that is impossible to determine without the radiographical analysis such as Cone Beam Computed Tomography (CBCT), ortophantomography, magnetic resonance imaging, to corroborate with clinical aspects, advising that, this could be the best way for the sake of patients benefit. Many treatments less aggressive have been proposed along the years, with a lower percentage of success against the traditional way, specifically surgical enucleation/marsupialization combined with peripheral osteotomy, in order to approach the variable behavior of nevoid basal cell carcinoma syndrome. The early diagnosis is considered the key to well manage this kind of disease, due to its aggressiveness, potential to become malignant, and in some cases, with a silent destructive pattern.

Keywords: Gorlingoltz syndrome, Odontogenic keratocyst, Palmar pits, Nevoid basal cell carcinoma syndrome