The main clinical manifestations of focal segmental glomerulosclerosis (FSGS) are proteinuria and nephrotic syndrome. FSGS is pathologically characterized by focal segmental distribution of glomerular sclerosis and podocyte foot process fusion, and is one of the main causes of end-stage renal disease. Due to the complex etiology and unclear pathogenesis of FSGS, its diagnosis and treatment still face many difficulties. This article reviews the current status of diagnosis and treatment of FSGS in recent years.