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. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.