There are few published reports on renal transplantation in patients with Down syndrome and renal failure. By means of a questionnaire sent to participating renal centers of the North American Pediatric Renal Transplant Cooperative Study, this study identified 14 Down syndrome patients who received renal transplants between January 1987 and November 1995. There were 6 males and 8 females between the ages of 6 and 21 years. Of the group, 8 patients were Caucasian and 6 were of African American ethnicity. Four patients had a primary diagnosis of obstructive uropathy, two had hypoplastic/dysplastic renal disease, two had focal segmental glomerulosclerosis, two had a diagnosis of chronic glomerulonephritis and four patients had unspecified diagnoses. Eight patients received cadaver kidneys whilst the other six had a living related transplant from a parent or sibling. Only one patient had received a prior kidney transplant, ten patients were on dialysis at the time they were transplanted and 4 patients were preemptively transplanted. Standard immunosuppression with Cyclosporine, Imuran and Prednisone was used. In addition, nine patients received induction therapy at the time of transplantation. Follow-up data revealed that 5 grafts (35.7%) had failed, two as a result of rejection and three due to patient death. At the time of data cutoff there were 3 deaths, one due to viral infection and the other two secondary to cardio-pulmonary complications.