It is an inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system, and bones. his shows a high level of penetrance and variable expressiveness, characterized by multiple basal cell nevi or carcinomas, odontogenic keratocysts, palmar and / or plantar pits, calcification of the falx cerebri, and is occasionally associated with internal malignancies. Early diagnosis is essential as it may progress to aggressive basal cell carcinomas and neoplasias. Here by we are reporting a case of gorlin goltz syndrome case here .