Buscher AK, et al. - Association of INF2 mutations with autosomal-dominant focal segmental glomerulosclerosis (FSGS) was reported in 2010, however, in the meantime, cases with more severe clinical courses have been described, including progression to end-stage renal disease (ESRD) during childhood. In this current investigation, it was discovered that there exists an association between INF2 mutations and familial proteinuric diseases - irrespective of the presence of FSGS and in the case of rapid disease progression. Hence, considering mutational analysis was recommended for patients with renal histology other than FSGS and severe renal phenotype.