Abstract: In autosomal dominant polycystic kidney disease(ADPKD), metabolic reprogramming occurs and aerobic glycolysis is significantly upregulated, providing sufficient energy and substrates for proliferation. Polycystins can regulate mitochondrial function directly and indirectly. The loss of functional proteins changes mitochondrial morphology and function, resulting in significant oxidative stress. Here, we reviewed and discussed the characteristics and mechanisms of energy metabolic changes and mitochondrial dysfunction in ADPKD. We also summarized the potential clinical targets for the intervention of energy metabolism in ADPKD.