Hereditary forms of nephrotic syndrome (NS) have been considered as infrequent disorders; however, 3–6% of the cases with NS have an affected sibling (1–3). Over the last decade, screening of large cohorts of pediatric patients presenting with steroid-resistant nephrotic syndrome (SRNS) for gene mutations has revealed the importance of genetic disorders in the pathogenesis of proteinuric glomerulopathies. At least 66% of the cases presenting with SRNS during the first year of life have an underlying genetic disease (4). In cases with infantile and juvenile SRNS, the overall proportion of genetic forms appears significantly lower, although the precise frequency remains unknown. Because autosomal recessive diseases may present as sporadic cases, the incidence of hereditary forms of NS is certainly underestimated. From a clinical perspective, most patients with hereditary SRNS will be resistant to immunosuppressive agents and do not experience relapse after transplantation (5–7).