Gardner Syndrome (GS) is an autosomal dominant genetic disorder characterized by the presence of multiple osteomas, intestinal polyps (IP), sebaceous and/or epidermoid cysts, along with other dental and visual abnormalities. In GS, the IPs present are predominantly adenomas and have, approximately, a 100% potential for change to malignancy, which generally occurs in individuals in the 20–40 year age range. The bone and dental signs of GS generally precede the gastrointestinal symptoms, reason for the importance of the dental surgeon in early diagnosis of IP. The bone lesions consist of craniofacial osteomas, which are benign osteogenic tumors caused by the proliferation of spongy or compact bone, being classified as endosteal and periosteal. Sometimes, they cause confusion with cemento-osseous lesions such as florid osseous dysplasia or familial gigantiform cementoma (FGC). The objective of this study is to present the case of a patient with GS treated at the Santa Casa de Misericórdia Hospital in Sobral, Ceará, Brazil, who had endosteal and periosteal osteomas. Diagnosis was made by means of clinical, imaging and histopathologic exams. The patient FLTS, currently aged 23 years, also presented sebaceous cysts and intestinal alterations (tubulovillous adenomas) and was submitted to four surgical procedures, during seven years, where only the lesions that caused esthetical and functional problems were removed. The patient evolved without complications, up to present date, and continues to be evaluated annually by the oral and maxillofacial and coloproctology surgery services, for the prevention of severe intestinal lesions.

Conflict of interest: None declared.