Gorlin–Goltz syndrome is an uncommon multisystemic disease with an autosomal dominant trait, with complete penetrance and variable expressivity, though sporadic cases have been described. The pathogenesis of Gorlin–Goltz syndrome is attributed to abnormalities linked to long arm of chromosomes 9 and loss or mutation of human patched gene. It is characterized by multiple Odontogenic Keratocyst of the jaws, multiple basal cell carcinoma and various skeletal deformities. Diagnosis is based on the various major and minor criteria. Here we present a detailed review article on the various clinicopathological aspects of this syndrome.