By using CRISPR/Cas9, we generated an ADTKD-UMOD mouse model carrying UMOD p.Tyr178-Arg186 del, the mouse equivalent of the human UMOD p.His177-Arg185 del in-frame deletion, one of the most prevalent mutations in human ADTKD-UMOD21. Here, UMOD is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.