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Juvenile nephronophthisis (NPHP), an autosomal recessive tubulointerstitial nephropathy, accounts for 10%–20% of renal failure cases in childhood. Treatment of patients with NPHP is symptomatic. Kidney transplantation is the treatment of choice when ESRD is established. We report herein our center's experience with kidney transplantation for children with juvenile NPHP.We retrospectively analyzed medical records of 142 renal transplant patients. We compared donor types, dialysis modality and duration, immunosuppressive treatment, acute rejection rate and outcomes of the patients with (group 1) and without (group 2) juvenile nephronophthisis.Seventeen (11.9%) had primary juvenile nephronophthisis diagnosis. Of the 17 NPHP patients (group 1), 5 (31.2%) received a cadaveric donor and 12 (68.7%) received a living related donor transplantation. Of the 125 without NPHP patients (group 2), 23 (17.6%) received a cadaveric donor and 102 (82.3%) received a living related donor. The mean age of the patients in group 1 was 149.56±50.33 months and 154.36±58.59 months in group 2 (P=.75). The mean follow-up period was 60.18±35.86 months in group 1, 46.35±30.51 months in group 2 (P=.09). There was no significant difference between groups in terms of dialysis modality and duration of dialysis and immunosuppressive treatment. Although the rate of acute rejection (11% vs 28.4%) and graft loss (5.25% vs 10.2%) were found lower in patients with NPHP, the difference was not statistically significant. Both groups had similar glomerular filtration rates post-transplant at 1 and 5 years (95.95±21.10 and 83.79±28.98 mL/min/1.73 m2 in group 1; 89.79±30.55 and 84.83±29.38 mL/min/1.73 m2 in group 2, respectively).We observed preserved graft functions for long periods and we can say that post-transplant prognosis is good among renal transplant recipients with juvenile NPHP.