OBJECTIVE Steroid-resistant nephrotic syndrome/focal segmental glomerulonephritis (FSGS) is the primary renal disease in approximately 10% of pediatric patients receiving a renal allograft. Risk factors for recurrence are a chronological age between 6 and 15 years at onset of the nephrotic syndrome and a rapid progression of the disease in the native kidneys leading to end-stage renal disease in less than 3 years. With rapid recurrence of FSGS and loss of the allograft, further renal transplants also carry a high likelihood of recurrence of nephrotic syndrome. RESULTS Different pathogenic factors have been discussed for the recurrence of proteinuria/FSGS in the transplanted kidney, especially the involvement of a proteinuric circulating factor. Treatment strategies are divided into two phases: induction of remission by plasma exchanges combined with high-dose intravenous or oral cyclosporine A; stabilization of remission by cyclophosphamide or rituximab, which showed promising results in several case reports. CONCLUSIONS No controlled studies have been performed yet to address the management of recurrent FSGS posttransplant. Complications related to the high-degree immunosuppression are not rare and should be regularly investigated. Therefore, the benefit: risk ratio for all immunosuppressive treatment strategies should be carefully evaluated for each individual patient.