Autosomal dominant polycystic kidney disease(ADPKD)is a single-gene inherited kidney disease characterized by the progressive formation of cysts,leading to increased kidney volume and decreased kidney function,and resulting in end-stage renal disease.The previous clinical treatment of ADPKD is mainly symptomatic supportive treatment,which cannot delay the progression of polycystic kidney disease.Therefore,an effective treatment plan for ADPKD is urgently needed clinically.In recent years,with the continuous in-depth research on the pathophysiological mechanism of ADPKD,the research and development of molecular targeting drugs for the pathogenic targets of ADPKD and related signaling pathways have made continuous breakthroughs.Currently,a variety of molecular targeting drugs for ADPKD have entered the stage of clinical trials.In this paper,the research progress of molecular targeting drugs of ADPKD was reviewed,in order to be helpful for the treatment of ADPKD and the development of new drugs.