PURPOSE: The aim of this study was to determine long-term results of intestinal transplantation in children with pseudo-obstruction, particularly when stomach and colon are not part of the allograft. METHODS: The authors conducted a case-record review of all children who underwent transplantation at our center for a primary diagnosis of pseudo-obstruction. Supplementary information was obtained from outpatient charts, computerized database, and telephone survey of parents. RESULTS: Six small bowel and 3 liver-small bowel transplants were carried out in 8 patients between 1993 and 1999. Median follow-up is 40 months (range, 13 to 73 months). Median age at transplantation was 2.7 years (range, 0.7 to 12.8 years). Median graft survival in this series is 15 months (range, 1 day to 71 months). Stomach and colon were excluded from all allografts. Two children died 5 and 368 days after transplant and 2 graft losses occurred in 1 patient. Two children had lymphoproliferative disease; both are alive with functioning grafts. Five survivors with functioning grafts receive full enteral feedings at home. Four of the 5 have had ileostomies closed, and 3 have normal bowel movements. CONCLUSIONS: Intestinal transplantation without stomach or colon provides children with chronic intestinal pseudo-obstruction with a good quality of life. The underlying disease poses special challenges in management.