Gorlin-Goltz syndrome – A case Report

Gorlin-Goltz syndrome is a rare autosomal dominant disorder that involves multiple organ systems, including the skin, skeleton and jaws. We report a case of young female with multiple odontogenic keratocysts, high arched palate, euryopia, palmer pits, solitary, pigmented nevus , areas of hyper-pigmentation in the upper eyelid of left eye, dorsal surface of hands, calcified diaphragma sellae, right 5 bifid rib & extensive bilamellar calcifications of the falx cerebri. Based on the combination of imaging and clinical findings the diagnosis of Gorlin-Goltz syndrome was made.