The phenotypes observed in patients with these 7 variants were UPJO, duplex collecting system and renal agenesis for FRAS1 variants, VUR, L-Duplex ureter, PUV and horse-shoe kidney (syndromic) for TNXB variants, VUR for FREM2 variant, PUV for CHD1L gene and VUJO with Duplex system for variant in SIX5 gene. The gene discussed is FRAS1; the disease is renal agenesis.