OBJECTIVE The pediatric patient with end-stage renal disease provides a great opportunity for the involvement of the pediatric urologist because of the high incidence of associated urologic anomalies. RESULTS Renal transplant remains the procedure of choice in the child with end-stage renal disease. The durability of survival of the graft is impacted by successful management of the genitourinary tract. CONCLUSIONS Preoperative, intraoperative, and postoperative evaluation is critical for the child with associated urologic disease and end-stage renal disease. Early and appropriate management may stabilize renal function and prolong the time to transplant. Due to the relatively long life expectancy of children compared with adults with end-stage renal disease, appropriate management may be of benefit to avoid the morbidity associated with renal replacement therapy. In certain children, the appropriate management and reconstruction of the genitourinary tract may allow for a planned and preemptive renal transplant, thus avoiding dialysis.