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We report here two cases of autosomal dominant polycystic kidney disease (ADPKD) with renal dysfunction that were treated with tolvaptan. Case 1 was a 47-year-old man with a glomerular filtration rate (GFR) of 17.0 ml/min/1.73 m 2 who received tolvaptan treatment (30 mg/day). After treatment, kidney pain was alleviated, and the estimated GFR (eGFR) decline improved from -9.84 ml/min/1.73 m 2 per year to -4.08 ml/min/1.73 m 2 per year, respectively. The rate of increase in total kidney volume was reduced from 18 % per year before treatment to 4 % per year following tolvaptan administration. Case 2 was a 44-year-old man with a GFR of 22.6 ml/min/1.73 m 2 , and the eGFR decline improved from -5.76 ml/min/1.73 m 2 per year before treatment to -3.12 ml/min/1.73 m 2 per year following tolvaptan treatment (30 mg/day). The rate of increase in total kidney volume was also decreased from 10 % per year before treatment to -7 % per year following tolvaptan administration. These results suggested that tolvaptan may be effective in impeding kidney function aggravation and kidney volume increase in ADPKD patients with advanced renal dysfunction.