Introduction: Gorlin syndrome is a rare disorder with different diagnostic criteria such as mul-tiple odontogenic keratocysts, basal cell carcinomas, palmar &plantar pits, frontal bossing and hypertelorism and calcification of falx cerebri.

Case Report: The case which is reported in the present study was a 27-years old woman re-ferred by a general dentist to oral medicine department of Hamadan dental faculty. On clini-cal and radiographic examination , multiple odontogenic keratocysts of jaws, multiple basal cell carcinomas, palmar pits and hypertelorism were obvious. The jaw cysts were treated with marsupialization and enucleation. The patient was referred to the dermatologist for pho-todynamic therapy.

Conclusion: Most disorders of Gorlin syndrome are slight, which usually do not threat the pa-tient’s life. The prognosis of this syndrome usually depends on the dermal tumor behavior.