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The occurrence of multiple odontogenic cysts in jaw bones is not a very common entity. Such occurrence should be ruled out for any association with the Gorlin–Goltz syndromes. It is important to diagnose the lesion and their associations with other features of the syndrome. Oral health care professionals play a very important role in early diagnosis and help in multidisciplinary approach to manage the condition for betterment of the patient. This paper highlights the multiple clinical manifestations of the syndrome with appropriate management strategies and recall protocols.

Nevoid basal cell carcinoma or Gorlin–Goltz syndrome is an autosomal dominant inherited disorder that results in numerous basal cell carcinomas (BCC) as well as a number of other facial and skeletal findings. The estimated prevalence varies from 1/57000 to 1/256000 with a male-to-female ratio of 1:1. Main clinical manifestations include multiple BCCs, odontogenic keratocysts of jaws, intracranial ectopic calcifications, and skeletal abnormalities affecting the ribs, vertebra, and skull. Management requires a multidisciplinary approach, with surgical removal of odontogenic keratocysts; surgery for BCCs should be indicated when the number of such lesions is limited. We report a case that was managed surgically, with a follow-up of more than 2 years having no signs of recurrence and relevant literature review.

Gorlin-Goltz syndrome is an uncommonly found rare disease which shows various possible diverse manifestations of multisystem anomalies, high degree penetration rate with variable expressiveness at least on three body systems. Cutaneous, skeletal, ophthalmological, neurological and reproductive systems are commonly affected in this syndrome. Multiple keratocysts of the jaws are the frequently developed early abnormality than any other. Hereditary autosomal dominant trait is explained as the causative factor for the development of the syndrome. In this case two major and seven minor anomalies were registered. The anomalies found in the patient were not categorized as serious life-threatening abnormalities excepting the multiple keratocysts which were treated by enucleation, curettage and Camoy′s solution application. An incidental detection of Gorlin-Goltz syndrome with multisystem anomalies of a 14-year-old female patient, who had reported for the swelling in the left body of the mandible, is described in this article.

Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS) is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.

Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon autosomal dominant inherited disorder characterized by numerous basal cell carcinomas, odontogenic keratocysts, and musculoskeletal malformation. It is occasionally associated with aggressive basal cell carcinoma and internal malignancies. It is about a muttisystemic process characterized by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and/or plantar pits and calcification of the falxcerebri. Hence, present a case report and a review of Gorlin-Goltz syndrome.