Familial Gorlin-Goltz Syndrome: A Case report

The Gorlin-Goltz syndrome (GGS), also known as Nevoid basal cell carcinoma syndrome(NBCCS) is a rare hereditary autosomal-dominant disorder characterised by multiple odontogenic keratocysts, basal cell carcinomas of skin as well as various neurological, ocular, skeletal, genitourinary disorders. Diagnosis of the syndrome is based on major and minor criterias first given by Evans et al (1993) which was later modified by kimonis et al (1997). Very few cases of this syndrome have been reported with familial background from India. We present a rare case report of Gorlin-Goltz syndrome with familial pattern affecting son and his mother.