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Gorlin's Goltz syndrome (GGS) is a rare autosomal dominant inherited condition. It in-volves many organs, but principally affects the skin, skeleton, endocrine and nervous sys-tems. It consists of the classic triad of basal cell carcinomas, multiple jaw cysts and skeletal deformities along with other defects. The authors report an unusual case of a fifteen year old male patient who reported with the complaint of swelling and mild pain in the lower right side of the jaw. Investigations revealed multiple cystic lesions in both upper and lower jaws which were found to be Odotogenic Keratocysts (OKC), presence of bifid rib and mul-tiple nevi. The clinico-pathological diagnosis was made as Gorlin- Goltz syndrome (GGS) since the present case exhibited three major criterias.
Nevoid basal cell carcinoma syndrome, Gorlin Goltz syndrome, Bifid ribs, Multiple odontogenic keratocyst cysts, Multiple Nevi, Palmar pits, PTCH gene