Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation can limit graft survival. Despite new immunosuppressive agents, the incidence of recurrence remains relatively high. To identify risk factors for recurrence and efficacy of treatment, we reviewed the outcome of 23 grafts in 16 children with FSGS who had undergone transplantation between 1985 and 2007 at La Paz Children's Hospital. Recurrence was 56.3% after the first transplantation. We did not find significant differences in age at diagnosis, age at transplantation, age at end-stage renal disease (ESRD), progression to ESRD, bilateral nephrectomy of native kidneys prior to transplantation, use of induction therapy or of different immunosuppressive regimens between patients with and without recurrence. Plasmapheresis (PP) was carried out in seven of nine patients who had suffered recurrence, achieving remission in six of them. One patient received high doses of cyclosporin (CsA) and plasmapheresis, attaining remission. Graft survival was lower (P = 0.043) in patients with FSGS than in those with other ESRD etiologies (first year 75% vs 91%; fifth year 44% vs 78%). Recurrence of FSGS limited graft survival (first year 66% vs 85%; third year 20% vs 68%) (P = 0.07). In our experience, PP can be effective in treating FSGS recurrence, although its effect on long-term graft survival seems more limited.