Indeed, mutations in genes encoding other components of the INVS compartment (INVS/NPHP2, NPHP3 and ANKS6/NPHP16) are known to lead to infantile NPH associated with enlarged cystic kidneys or to kidney cystic dysplasia associated with congenital heart defects and situs inversus [9, 11–13]. Here, NPHP3 is linked to Renal cyst.