Focal segmental glomerulosclerosis (FSGS) is a clinicopathological syndrome characterized by focal segmental glomerulosclerosis and podocyte fusion. The main clinical manifestations are massive proteinuria and nephrotic syndrome (NS). It mostly is hormone resistant and often progresses to end-stage renal disease. It is one of the main diseases that progress to endstage nephropathy in children. In recent years, the detection rate of FSGS by renal biopsy is increasing worldwide. This article reviews the progress in epidemiology of FSGS.