A 46-year-old Jordanian man was seen in ENT clinic complaining of prog
ressive left nasal obstruction and hearing loss for the last five year
s with a history of multiple surgical removal of dental cysts. Clinica
l and radiological investigation revealed a cystic mass occupying the
left maxillary sinus protruding to the nasal cavity, calcification of
the fair cerebri and bifid ribs. On these findings Gorlin-Goltz syndro
me was confirmed. The case is presented and the literature reviewed.