Abstract: The prevalence of autosomal dominant polycystic kidney disease(ADPKD) is 1‰ to 2‰, belonging to rare diseases. ADPKD is mainly manifested as gradually developing bilateral renal cysts, progressively increasing renal size, and gradually reduced renal function. PKD1 mutation accounts for about 81%; PKD2 mutation accounts for about 10.5% to 22%. Vasopressin and cyclic adenosine monophosphate(cAMP) signaling pathways play an important role in the development of ADPKD cysts. In recent years, the Mayo risk assessment model and predicting renal outcome in polycystic kidney disease(PROPKD) score, which are good prognostic models for ADPKD, have become important evidences for clinical decision-making. Tolvaptan, which inhibits cAMP pathway by antagonizing vasopressin receptor, has become the first specific treatment for ADPKD. Tolvaptan could effectively inhibit the growth of total renal size, and protect renal function. Long-term safety of the drug still needs further study.