Polycystic kidney disease (PKD) is mainly classified into autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). ADPKD is the most common human hereditary kidney disease, ranking the fourth cause for end-stage renal disease. In recent years, PKD registration databases developed rapidly in developed countries, including clinical and genetic information, registration of therapy, specimens, and research information. Now it is urgently needed for our country to establish a PKD registration database so as to provide data for further study on the pathogenesis of ADPKD, block disease heredity, and delay the progression of ADPKD.