HNF1B-nephropathy is recognized to represent an autosomal dominant syndromic disorder comprising kidney injury (kidney cysts, glomerular tufts, primitive tubules, irregular collecting systems, oligomeganephronia, enlarged kidney pelvises, abnormal calyces, small, single or horseshoe kidney), diabetes mellitus (maturity onset diabetes of the young type 5 (MODY5)), elevated cholestatic liver enzymes, hyperuricemia, pancreatic and genital tract malformations (vaginal aplasia, rudimentary uterus, bicornuate uterus, cryptorchidism, epididymal cysts and atresia of the vas deferens) [11]. Here, HNF1B is linked to Nephropathy.