The recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation has a potentially detrimental impact leading to the loss of renal function. Although, plasmapheresis (PF) and rituximab are commonly recommended the treatment is still a matter of debate. We report our single-center experience to assess the outcome of the renal transplantation in children with FSGS. Medical records of 10 (F/M: 4/6) renal transplanted patients with FSGS were evaluated. Among 10 grafts 7 were from living related and 3 from deceased donor. The original diagnosis of FSGS as well as recurrences was biopsy-proven in all patients. All patients treated with calcineurin-based immunosuppressive therapy. PF was done at days -3,-2,-1 and 5 consecutive days following transplantation for all living related donor transplanted patients. Patients with deceased donation had only post-transplant PF. The mean age was 12.6±4.7 years. The mean duration of follow-up was 23.1±16.2 months. Two patients with hyperacute rejection were followed-up for less than 1 month. One of them had biopsy-proven humeral hyperacute rejection while the second was implicating recurrence of FSGS. Post-transplant recurrence of FSGS was confirmed in 3 (33%) patients and all of them were treated with pre and post-transplant PF while 2 have also received rituximab. Remission was obtained in 1 of 2 patients that have received rituximab while the other had responded partially. The one who did not receive rituximab had a graft loss at the 2nd month of transplantation. Recurrence of FSGS in the transplanted kidney is a severe condition associated with graft loss. New therapeutic regimens and the efficacy of rituximab and PF should be evaluated in prospective studies with large groups.