Treatment in children with end-stage renal disease, especially those with significant bladder dysfunction, is difficult. A high-pressure and low-capacity bladder is a major risk factor for a transplanted kidney. Cystoplasty can protect the kidney allograft by the reduction in the intra-bladder pressure and producing an appropriate capacity. The aim of this study was to evaluate the outcome of kidney transplantation in the children with cystoplasty in comparison with the control group. A total of 43 children with bladder dysfunction in urgent need of cystoplasty were enrolled in the study and were compared with the control group regarding the acute and chronic rejection rates, survival of the transplanted kidney, surgical complications, and febrile UTI.The frequency of febrile UTI and chronic rejection was significantly higher in the group of the patients with cystoplasty. Also, graft loss was much more frequent in these patients (30% vs 20%) but this difference was not statistically significant. In the patients with cystoplasty, the survival rate of the transplanted kidney was 92%, 73%, 58%, and 45% at the 1st, 3rd, 5th, and 7th postoperative year, respectively. In the control group there rates were 94%, 87%, 81%, and 75%, respectively (P<.05). According to our findings, the survival rate of the kidney is significantly lower in the children with cystoplasty, and this could be due to the higher prevalence of chronic rejection and febrile UTI in this group.