In the current study, we generated heterozygous PKD1 KO cloned pigs harboring the unique mutation c.152_153insG (PKD1insG) in the first exon of PKD1. The resulting PKD1insG/+ cloned pigs displayed characteristics of an ADPKD model, including (i) neonatal renal cyst formation, (ii) progressive cyst development during animal growth, and (iii) sustained fertility after sexual maturation. This evidence concerns the gene PKD1 and Renal cyst.