Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is a rare condition characterized by varied clinical manifestations like multiple Basal Cell Carcinomas (BCC), multiple Keratocystic Odontogenic Tumours (KCOT), palmar and/or plantar pits and ectopic calcification of the falx cerebri, which are considered as the major criteria for diagnosis. The occurrence of jaw manifestations makes it an important diagnostic problem for oral and maxillofacial surgeons and often clinicians encounter this aspect which finally leads to the diagnosis of this syndrome. This paper reports a case of NBCCS and provides an overview on the diagnosis and management of this enigmatic entity.
Keywords: Gorlin-Goltz syndrome; Keratocystic odontogenic tumour; Nevoid basal cell carcinoma syndrome.