BACKGROUND: Restorative proctocolectomy with IPAA is an optimal surgical treatment for patients with familial adenomatous polyposis and therapy resistant ulcerative colitis, few data are available on long-term results in patients who have undergone this operation at a young age. OBJECTIVE: The aim of this study was to investigate long-term functional outcome, quality of life, body image, and sexual function after restorative proctocolectomy with IPAA in young patients with familial adenomatous polyposis or ulcerative colitis. DESIGN, SETTINGS, AND PATIENTS: The study consisted of a retrospective review of medical records and questionnaire follow-up of 26 consecutive patients with familial adenomatous polyposis or ulcerative colitis who had undergone surgery between January 1992 and October 2008 at the Maastricht University Medical Center, were aged 10 to 24 years at the time of surgery, and had at least 1 year of follow-up after surgery. MAIN OUTCOME MEASURES: We reviewed medical records from an institutional database regarding surgical procedures and short- and long-term complications. At the end of 2009, validated questionnaires covering bowel function (Colorectal Functional Outcome Questionnaire), quality of life (Short Form-36 and Gastrointestinal Quality of Life Index), body image and cosmesis (Body Image Questionnaire), and sexual function (International Index of Erectile Function for men and Female Sexual Function Index for women) were mailed to patients. RESULTS: Median age at surgery was 18 years, and median follow-up was 12.5 (range, 2-18) years. Long-term colorectal complications occurred in 23 patients (88%), and were directly related to the surgery in 20 patients (77%). Five patients needed pouch excision. The questionnaire response rate was 88%. Bowel function, quality-of-life, and body image and cosmesis scores were all lower in patients than in historical normal control populations. Men did not report impotence or retrograde ejaculation, but 50% of women reported sexual dysfunction. LIMITATIONS: This was a retrospective study in a small number of patients from a single institution with no comparison groups. CONCLUSIONS: Restorative proctocolectomy with IPAA can be performed in young patients with an acceptable functional outcome, but at the cost of relatively high complication rates, poor body image and cosmesis, and a high rate of sexual dysfunction in women. Because young patients undergoing this surgical procedure may experience negative long-term effects, surgeons should be aware of all potential consequences, inform patients as to what to expect, and ensure long-term follow-up to deal with long-term complications.