At least five (45%) and possibly nine (82%) of the 11 families studied had disease-associated heterozygous variants consistent with a coexistent genetic kidney disease (autosomal dominant (AD) and X-linked (XL) Alport syndrome, ADTKD-<i>HNF1B</i>, and possibly Dent disease, Focal and Segmental Glomerulosclerosis (FSGS), and CHARGE syndrome). Here, HNF1B is linked to autosomal dominant medullary cystic kidney disease with or without hyperuricemia.