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Gardner's syndrome, phenotypic variant of the familial adenomatous polyposis (FAP), is characterized by the triad: colorectal polyposis, desmoid fibromas and sebaceous cysts and dento-maxillary lesions made of highly polymorphic, such as osteomas, odontomas, supernumerary teeth and impacted teeth. The major problem of this disease with dominant autosomal transmission lies in the polyps which are then transformed into adenocarcinomas from the fourth decade. The dental surgeon can be brought to evoke the syndrome and to play a role in the early detection. This role is due to dental lesions that can be observed from the young age and precede the gastrointestinal damage. The purpose of this work is to relate a case report of 15 year-old girl whose clinical picture prompted us to search for the Gardner syndrome. The diagnosis was confirmed by gastrointestinal examination. The case control also allowed to spread the investigations to all the family and to detect numerous affected members. [ABSTRACT FROM AUTHOR]