Isfahan University of Medical SciencesDental Research Journal1735-33279120120218Gorlin-Goltz syndrome903903ENPriya ShirishJoshiDepartment of Oral Pathology and Microbiology, Vasantdada Patil Dental College and Hospital, Kavalapur, Sangli, India. sangeetakov@yahoo.co.inVijayDeshmukhSomeshwarGolgire20120218Gorlin-Goltz syndrome is an uncommon autosomal dominant inherited disorder, which ischaracterized by multiple odontogenic Keratocysts and basal cell carcinomas, skeletal, dental,ophthalmic, and neurological abnormalities, intracranial ectopic calcifications of the falx cerebri,and facial dysmorphism. Pathogenesis of the syndrome is attributed to abnormalities in the longarm of chromosome 9 (q22.3-q31) and loss or mutations of human patched gene (PTCH1 gene).Diagnosis is based upon established major and minor clinical and radiological criteria and ideallyconfirmed by deoxyribo nucleic acid analysis. We report a case of a 9-year-old girl presentingwith three major and one minor feature of Gorlin-Goltz syndrome. Radiologic findings of thesyndrome are easily identifiable on Orthopantomogram, chest X-ray, and Computed tomographyscans. These investigations prompt an early verification of the disease, which is very important toprevent recurrence and better survival rates from the coexistent diseases.Key Words: Calcification of falx cerebri, fused ribs, Gorlin-Goltz syndrome, odontogenickeratocyst