The Gorlin-Goltz syndrome (GGS) or (the nevoid basal cell carcinoma syndrome-NBCCS) is a rare autosomal dominant and multiple organ syndrome. The common manifestations include multiple odontogenic keratocysts in the jaws, multiple basal cell nevi on the skin with an early age onset, palmar pits,bifid or splayed ribs, high arched palate, euryopia , areas of hyper-pigmentation in the upper eyelid of left eye, dorsal surface of hands, calcified diaphragm sellae, calcifications of the falx cerebri. Although all the manifestations may not be present in all the patients. In this case report we report only case of gorlin-goltz syndrome in jammu region in young 20 year old female patient and are reviewing the features of the Gorlin-Goltz syndrome.

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