PURPOSE: This study was designed to evaluate the results of restorative proctocolectomy with distal rectal mucosectomy and ileal pouch-anal anastomosis in children. METHODS: This study is a retrospective review of 100 consecutively referred children (<18 years old) who underwent reconstruction with a J-pouch of ileum and preservation of the transitional anorectal epithelium by the same two-surgeon team. Temporary diverting ileostomy was used. The main outcome measures were daytime and nocturnal fecal continence, bowel movements per day, and complications including pouchitis, ileoanal stricture, or postoperative small-bowel obstruction. RESULTS: Average age of the 100 children (48 males/52 females) was 13.2 years (range, 2.95-17.99). All 25 children with familial adenomatous polyposis had proctocolectomy and reconstruction performed simultaneously. Of 75 children with ulcerative colitis, 50 (67%) had their colectomy followed by reconstruction after an interval ranging from 2 months to 4.4 years. Median postoperative follow-up was 2.6 years. Daytime fecal continence was achieved in 98 children, although 4 reported rare accidents. Nighttime continence was achieved in 93 children, of whom 14 reported rare accidents. The average frequency of bowel movements was 5.43/day (+/- 2.22). Only one child with polyposis had pouchitis. Of 75 children with ulcerative colitis, 35 had symptoms consistent with pouchitis; of these 35 children, 10 required prolonged treatment. The most frequent postoperative complication was ileoanal stricture requiring operative dilatation and/or anoplasty (18 children). Bowel obstruction requiring surgery occurred in 18 children. One child eventually required pouchectomy for probable Crohn's disease. CONCLUSIONS: Excellent results can be achieved with restorative proctocolectomy in children with respect to fecal continence and stool frequency. However, with ulcerative colitis, a substantial risk of pouchitis remains.