Snapshot A 23-year-old man presents with problems involving his teeth, bones, and vision. There is a family history of tumors of the thyroid and adrenal glands. Clinical evaluation reveals supernumerary teeth, long-bone osteomas, and increased retinal pigmentation. Introduction Subtype of familial adenomatous polyposis (FAP) FAP plus bony and soft tissue tumors login to view 6 more bullets ETIOLOGY Genetics login to view 3 more bullets Presentation Symptoms/physical exam login to view 16 more bullets IMAGING Endoscopy login to view 1 more bullet Radiography login to view 1 more bullet STUDIES Genetic testing login to view 1 more bullet Differential Diagnosis Peutz-Jegher syndrome Lynch syndrome DIAGNOSIS Based on endoscopy for polyposis, clinical exam, family history, and genetic testing Treatment Prophylactic colectomy as in FAP Routine screening for extracolonic tumors login to view 1 more bullet Complications Untreated growth of desmoid tumors login to view 2 more bullets Prognosis Almost normal with colonic screening and colectomy