Gorlin Goltz syndrome

Gorlin syndrome, also known as Gorlin-Goltz syndrome, basal cell nevomatosis, is a hereditary disease that is transmitted dominant mode, with complete penetrance and variable expressivity. This rare disorder is characterized by a series of developmental abnormalities and a predisposition to various cancers and present many clinical manifestations such as skeletal, dental, gynecological, ophtalmological, cardiac and renal anomalies. The complexity of clinical manifestations has led to the establishment of specific criteria to simplify diagnosis and has required a multidisciplinary approach for the treatment and follow-up of patients.The aim of this work was to present clinical manifestations and the diagnostic approach of Gorlin-Goltz syndrome and detail the treatment especially the role of the dentist to carry out these patients.