We describe a patient who presented with nephrotic syndrome and progressive renal failure with biopsy findings of focal segmental glomerulosclerosis (FSGS). He progressed rapidly to end-stage renal disease (ESRD) and remained hemodialysis dependent for five months despite high dose prednisone therapy. Initiation of plasmapheresis and low dose oral cyclophosphamide resulted in the prompt return of urine output and renal recovery. He remains off dialysis with stable renal function (creatinine clearance = 40 mL/min) two years later. This is the first report of late rescue from apparent ESRD due to FSGS with combined plasmapheresis and low dose oral cyclophosphamide.