The authors presenta retrospective analysis of forty-four patients with familiar adenomatosis polyposistreated at the School of Medicine of Ribeirão Preto Hospital and Clinics University of São Paulo, from January 1991 to July 2005. Epidemiologic(age and gender) and genetic aspects were investigated as well as main symptoms,personal history and surgical treatment outcome. Data obtained were comparedto the available literature. Our results show that 31 patients were male and13 female, with average age of 32 years-old (14 to 60 years-old). The main symptomswere intestinal bleeding (62.5 %) and intestinal habit alteration (60 %). Lessfrequently, patients showed abdominal pain (45 %) and loss of weight (30 %).Familiar adenomatosis polyposis was reported by 67.5 % of the patients and relativeswith neoplasms (intestinal and extra-intestinal) were referred by 62.5 % ofthe patients. By the time of the polyposis diagnosis, 32.5 % of the patientshave already been diagnosed for colon cancer, with average age of 39 years old.Surgical treatment was performed in 95.4 % of the patients, total proctocolectomywas performed in 35.7 % (9 cases with ileal J- pouch and 6 with permanent ileostomy)and total colectomy with ileo rectal anasthomosis in 59.2%. From all patientsevaluated, 57 % are still under follow up, 7 % died and 27 % gave up treatment.