DOI: 10.36347/sasjs

Pages: 204-208

Multiple KCOT’s (Keratocystic odontogenic tumors) of maxilla and mandible are rare and most of the times they are associated with a syndrome. Gorlin Goltz or Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal dominant systemic disease associated with multiple KCOTs of jaws along with cutaneous, ophthalmologic, skeletal, neurologic and genital abnormalities. KCOTs associated with Gorlin Goltz Syndrome reportedly have high recurrence rates of up to 82% and are the presenting complaint in many cases of this syndrome. Hence maxillofacial surgeons play a crucial role in early identification of the disease. Although life expectancy is not significantly altered, early diagnosis may prevent morbidity due to existing complications improving the quality of life. Our aim is to report experience with 5 cases of Gorlin Goltz Syndrome in two related families.