Background and Objective: Cystinosis is a rare inherited disease that leads to renal failure. Fanconi syndrome is the major renal involvement in cystinosis patients. Renal transplantation is the treatment of choice in cystinosis children with end-stage renal disease (ESRD).  The study aimed to assess the outcome of renal transplantation in Iranian children with cystinosis.

Methods: This retrospective study is a follow up of 21 cystinosis children transplanted in Labafinejad Hospital, Tehran, Iran.

Results: Three cystinosis patients involved by primary non-function because of graft vein thrombosis and/or severe acute tubular necrosis. The remaining cystinosis patients had excellent graft survival rate and only one patient lost the graft 3 years post-transplant due to noncompliance. The graft survival rate after excluding the patients with primary non-function was 100%, 94%, 94% and 94% at 1,3,5 and 10 years after transplant. The mean serum creatinine in patients with functioning graft 10 years after transplant was 1.6 mg/dl.

Conclusion: We showed that cystinosis patients had good graft function in long term after transplantation.