Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy that progresses to end-stage renal disease in children. It is also notorious for frequent recurrence after transplantation. The recurrence of FSGS after transplant leads to the loss of the grafts in nearly half of the recurrent cases, especially in those who did not respond to treatment. Remission can be achieved in majority of the recurrent patients as long as the treatment is initiated early enough (＜48 hours after onset). Since recurrence typically manifests as massive proteinuria, usually within a few days after the engraftment, close monitoring of proteinuria and prompt treatment on recurrence is advisable. Plasmapheresis to remove the ‘circulating factor’ been used as a mainstay of treatment of the recurrence; on each session, one to two times of plasma volume is exchanged, and the treatment is continued for 8∼12 sessions or until remission is achieved. High dose cyclosporine is another arm of commonly practiced treatment with good result. Recently, depletion of B cells by rituximab has been tried as another option of treatment of recurrence, with excellent response by some and no response by others. Again, prompt administration of the treatment seems critical to achieve remission. The efficacy of pre-emptive, prophylactic plasmapheresis before transplantation is controversial. In summary, the recurrence of primary FSGS is observed in more than half of the children after transplantation. Prompt initiation of treatment would improve the survival. To improve the prognosis of recurrent primary nephrotic syndrome/FSGS, close monitoring and multidisciplinary approach is required.