Children with the prune belly syndrome are at high risk for renal failure. The effect of the prune belly syndrome on the outcome of renal transplantation was evaluated in a retrospective study in which 8 transplant recipients with this syndrome were randomly matched with 13 control, nondiabetic transplant patients. The parameters evaluated were patient and graft survival, renal function and interval until transplantation. The patients were matched by computer for age at transplantation, date of transplantation, immunosuppressive therapy used and type of donor. Data were analyzed by the Gehan test. There was no statistically significant difference in patient deaths (1 versus 3), graft survival (75 versus 69%) or graft function between patients with the prune belly syndrome and controls. Patients with the prune belly syndrome waited a shorter interval for transplantation than did controls (no statistically significant difference) because the distensible abdominal wall characteristic of the syndrome permits placement of an adult kidney in a young child. The prune belly syndrome did not adversely affect the outcome of renal transplantation in these patients.