ADPKD is a genetically and phenotypically heterogeneous disorder, with approximately 80–85% of cases caused by variants in PKD1, and 15–20% by variants in PKD2. Recently, monoallelic IFT140 variants, and rare heterozygous variants in GANAB, DNAJB11, ALG8, ALG9, and ALG5 were shown to cause milder ADPKD-spectrum disorders[2]. This evidence concerns the gene PKD1 and autosomal dominant polycystic kidney disease.