[Gardner syndrome. Maxillofacial management of an extremely advanced form]

Gardner's syndrome is a very rare hereditary disease resulting from an embryo genesis disorder involving all three ectodermal, mesodermal and endodermal layers. Generally non-digestive tract signs disclose the syndrome. Underlying silent digestive anomalies should be investigated. Endodermal anomalies may have a fundamental effect on prognosis. Manifestations in our case were severe, with ectodermal and mesodermal anomalies occurring late. Maxillofacial management was required because of the critical esthetic and functional implications.