Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease. While biallelic variants affecting IFT140 are responsible for Mainzer-Saldino syndrome (characterized by severe ciliopathy causing skeletal abnormalities, kidney disease and cysts), monoallelic loss of function (LoF) variants have been recently reported as an important cause of ADPKD beyond PKD1/2 genes. Herein, we report six non-family-related cases of monoallelic IFT140 LoF variants, identified from 1340 exomes sequenced for nephrological indications in our local database.