Clinically, 61–90% of HNF-1β mutation carriers exhibit structural or functional renal abnormalities(136), and their pathological phenotypes show significant heterogeneity, mainly covering renal ciliopathy with anomalies of the kidney and urinary tract (RCAD)(26), glomerulocystic kidney disease (GCKD)(137), autosomal dominant tubulointerstitial kidney disease (ADTKD)(138), and congenital anomalies of the kidney and urinary tract (CAKUT)(139). Here, HNF1B is linked to congenital anomaly of kidney and urinary tract.