BackgroundPrimary focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome. AimsThe pathogenic steps leading to primary FSGS are still obscure, although evidence suggests that circulatory factor(s) are involved in the onset of disease. ResultsRecent technical advances allow the analysis of miRNA expression in tissues and body fluids, leading to reports of miRNAs involved in the molecular mechanisms of FSGS-aetiopathogenesis. Moreover, investigations have also highlighted miRNAs that might serve as biomarkers for primary FSGS. Discussion/ConclusionsThe aim of this review was to summarize reports showing a direct relation between miRNAs and primary FSGS. In addition, the impact of identified miRNAs on treatment response, prediction of the disease onset as well as the regulation in different disease activities is summarized.