Background: Idiopathic nephrotic syndrome (INS) is the most frequent glomerular disease affecting children. Its pathogenesis is not completely evident, it is probably due to immunological disturbance that increases cytokines production and alters the glomerular permeability. Interleukin 13 (IL-13) has been implicated in INS pathogenesis by changing the permeability of the glomerular basement membrane and inducing proteinuria. Objective: To investigate the association between rs20541 (R130Q) single nucleotide polymorphism (SNP) in IL-13 gene, idiopathic nephrotic syndrome susceptibility and steroid treatment response in Egyptian children. Methodology: This cross sectional case-control study was performed on 50 INS children aged 2-15 years following up in the Nephrology Unit of the Pediatric department at Benha University Hospitals and 50 healthy age and sex matched children as controls. All candidates were subjected to clinical evaluation. Genotyping of the selected SNP was performed using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Results: A highly significant frequency of GA genotype, p=0.017, OR= 1.933 (1.126-3.319) and AA genotype, p=0.001, OR=6.660 (2.283-19.433) as well as A allele, p