In case-2, a heterozygous female patient (NM_0.33380.3_COL4A5:c.4821+3A>G; initially reported as: IVS49+3A>G), who co-inherited the pathogenic recessive podocin variant NPHS2:p.Arg229Gln, presented very early with severe disease, with biopsy-proven focal segmental glomerulosclerosis. Here, NPHS2 is linked to focal segmental glomerulosclerosis.