Abstract
A 46-year-old Jordanian man was seen in ENT clinic complaining of progressive left nasal obstruction and hearing loss for the last five years with a history of multiple surgical removal of dental cysts. Clinical and radiological investigation revealed a cystic mass occupying the left maxillary sinus protruding to the nasal cavity, calcification of the falx cerebri and bifid ribs. On these findingsGorlin-Goltz syndrome was confirmed. The case is presented and the literature reviewed.