Recent advances in next-generation sequencing (NGS) technology have led to elucidate the molecular etiology of childhood-onset focal segmental glomerulosclerosis (FSGS) and nephrotic syndrome (NS) and helped unravel the pathogenic pathways that provide direction for precision medicine. Genes known to cause childhood-onset FSGS and NS, mostly found in patients with steroid-resistant who do not respond to initial glucocorticoid treatment. Now it is widely accepted that about 30% of patients with steroid-resistant NS can be genetically diagnosed through NGS. Patients with genetic FSGS and NS usually experience proteinuria and/or edema at an early age and response poorly to conventional immunosuppressants compared to patients without genetic mutations. A significant proportion of children with genetic FSGS and NS progress to chronic kidney disease and leads to kidney failure. In 2020, IPNA published clinical practice recommendations for the diagnosis and management of children with steroid-resistant NS. They recommend withholding calcineurin inhibitors and stopping prednisolone/prednisone treatment in patients with evidence for a monogenic form of steroid-resistant NS. Meanwhile, a recent discovery of genes associated with mitochondrial podocytopathies suggest that patients with pathogenic mutations in this group of genes are more likely to benefit from dietary coenzyme Q10 (CoQ10) supplementation. To date, five genes are known to cause primary CoQ10 nephropathy, PDSS1, PDSS2, COQ2, COQ6 and COQ8B. This session covers current knowledge of primary CoQ10 nephropathy, current and future therapies.