Multiple intestinal polyps (75% malignant transformation), desmoid tumors, epidermal cysts, fibromas, tooth impactions, jaw osteomas, retinal hypertrophy, periampullary cancers, thyroid cancer, and benign bone tumors; autosomal dominant condition. Onset of symptoms usu. occurs by 20 years of age. Treatment is total or subtotal colectomy with fulguration of remaining rectal polyps and surveillance upper endoscopy at 3Ð5-year intervals.