Background: Nevoid basal cell carcinoma syndrome (NBCCS), popularly known as Gorlin Goltz syndrome, is an autosomal dominant hereditary disorder encompassing various developmental abnormalities and neoplasms. It is frequently detected by maxillofacial surgeons owing to the presence of jaw cyst, however a multidisciplinary approach is never taken.

Case Presentation: Case report of a patient with a characteristic habitus, in which Odontogenic Keratocyst served as an alarm bell for suspecting a syndromic condition, Gorlin Goltz Syndrome. The patient was treated for her Odontogenic Keratocyst and underwent a complete physical check-up from various specialties for early diagnosis of other lesions and existing comorbidities.

Conclusion: Gorlin & Goltz^[1] described the classical triad composed of multiple basal cell carcinoma, odontogenic keratocyst (OKC) in the jaws and bifid ribs that characterized the diagnosis of this syndrome. It has been our attempt to provide the patient with a holistic, multidisciplinary approach.

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Dr Aditi Bardia Ghorawat

Post Graduate Student, Department of Oral and Maxillofacial Surgery, Sri Aurobindo College of Dentistry and P.G. Institute, Indore