In 2017, the World Health Organization (WHO) reclassified the keratocystic odontogenic tumor back into the odontogenic cysts category (OKC)(3), however this is still a controversial matter, since 85% of OKCs related to the Nevoid basal cell carcinoma syndrome (NBCCS) and 30% of non-syndromic cases present mutations in the PTCH gene (6). This evidence concerns the gene PTCH1 and nevoid basal cell carcinoma syndrome.