Focal segmental glomerular sclerosis (FSGS) is a common glomerular disease in children. Its clinical manifestations are mostly nephrotic syndrome. Some children do not respond well to glucocorticoids treatment, the first line treatment, and eventually progress to end-stage renal disease. Therefore, a variety of new drugs for the treatment of FSGS have been gradually developed and achieved certain efficacy. This paper reviews the treatment progress of primary FSGS in children in order to provide the basis and ideas for treatment and scientific research