Gorlin Goltz Syndrome, or Nevoid Basal Cell Carcinoma Syndrome, is an autosomal dominant genetic disease characterized especially by the manifestation of basal cell carcinomas in the skin and keratocysts in the jaws. Additionally, patients may present with several other manifestations, such as bifid ribs, hypertelorism and calcification of the falx cerebri. The diagnosis has physicians and dentists as protagonists in the investigation and involves a good anamnesis associated with a detailed physical examination, imaging, anatomical and histopathological exams and, if possible, the identification of the PTCH1 gene, which covers approximately 85% of patients. The present study aims to present an up-to-date integrative literary review of the Gorlin Goltz Syndrome, highlighting the care and management of the case of a syndromic patient treated at a stomatology clinic, carrying a series of major and minor findings of the disease.

Immunohistochemistry have had a huge impact on oral and maxillofacial pathology diagnosis. As a method it determines distribution and amount of certain cellular molecules via specific antigen-antibody reaction. Whereas in most cases a definitive diagnosis is achieved based on detailed hematoxylin and eosin cytomorphological analysis, along with clinical and radiological features, some challenging and equivocal neoplasms need to be further assessed with immunohistochemistry. This article reviews immunohistochemistry technique fundamentals and its role and relevance in the diagnosis of common oral and maxillofacial lesions encountered in  daily practice.