ABSTRACT
Rationale: Gorlin-Goltz syndrome (GGS) is an autosomal dominant disorder and is associated with multisystem involvement, multiple cysts, neoplasms and other developmental anomalies. The purpose of the study was to highlight the incidental findings of GGS and to lay emphasis on its early diagnosis. Patient Concerns: Two patients complaining of pain, swelling and at times pus discharge from the oral cavity were reported with a coincidental finding of odontogenic keratocysts and positive family history. Diagnosis: Upon thorough examination, a diagnosis of GGS was made. Treatment: The patients were managed by enucleation and chemical cauterisation using Carnoy's solution and were maintained on follow-up semi-annually. Outcomes: Both patients showed no signs of recurrence post six months follow-up. Lessons: The role of an oral and maxillofacial surgeon is of utmost importance in the early diagnosis of this syndrome to render good quality of life to these patients.
