Patients suffering from familial adenomatosis polyposis develop multiple pre-malignant gastrointestinal polyps and are at high risk of developing colon cancer. In addition extra-intestinal manifestations are observed frequently. The combination of extra-intestinal manifestations and familial adenomatosis polyposis is named Gardners syndrome. An early diagnosis of this disease is important because it could mean a better prognosis for the patient. This review describes the oral and maxillofacial symptoms of FAP, and its potential implications for dental treatment.