In this study, we report the cases of two children with significant proteinuria due to de novo nonsense mutations of the <i>TRIM8</i> gene.<h4>Case presentation</h4>Case 1 was a 7-year-old girl who presented with proteinuria and developmental delay, and her renal biopsy showed FSGS. This evidence concerns the gene TRIM8 and focal segmental glomerulosclerosis.