ABSTRACT
The nevoid basal cell carcinoma syndrome is a rare and autosomal dominant inherited disease with well-defined characteristics, summarized by Gorlin and Goltz in 1960. In the head and neck region, cerebral calcifications, Basal Cell Carcinoma [BCC] and multiple Odontogenic Kerato Cysts [OKC] of the jaws are the predominant findings. Odontogenic keratocysts with high rate of recurrency and basal cell carcinoma of the facial skin are important to dentists. In this article we present a 23 year old male with multiple recurrent cysts of the jaws who diagnosed to have Gorlin syndrome. The patient also has polydactily, palmar and plantar pits, bifid ribs, hypertelorism, calcification of falx cerebri and missing of four wisdom teeth. Diagnosis, treatment and follow up of this case are discussed