Gorlin Goltz syndrome, also known as basal cell nevus syndrome, is a rare genetic disorder caused by a mutation in the PTCH gene. It is characterized by multiple basal cell carcinomas, odontogenic keratocysts of the jaws, skeletal abnormalities such as bifid ribs and calcification of the falx cerebri. The incidence varies from 1 in 57,000 to 1 in 256,000. Management requires a multidisciplinary team and focuses on complete surgical removal of odontogenic keratocysts, orthodontic treatment to preserve dental and facial structure, and genetic counseling due to its autosomal dominant inheritance.