Abstract: Kidney transplantation (KT) in children with end-stage renal disease and an abnormal bladder poses a complex management challenge. Ureterocystoplasty (UC) has been previously reported in older children with non-compliant bladders, but the timing and technique of repair are controversial. This case reports the youngest patient, a 20-month-old boy to undergo successful single-stage UC and living-related KT. UC was performed because of a fibrotic, non-compliant bladder. A temporary vesicostomy was placed to provide adequate drainage in the presence of urethral stenosis. The patient developed a single episode of pyelonephritis within the first six months post-operatively, but there were no other urologic complications. At 13 months, the renal function is excellent with a mean glomerular filtration rate of 100 mL/min/1.73 m2 and no clinical evidence of rejection. This case demonstrates that simultaneous UC and KT can be safely performed even in infants with non-compliant bladders and renal failure.