A 59-year-old Japanese woman was admitted for evaluation of muscle weakness. Autosomal dominant polycystic kidney disease had been diagnosed at the age of 47 years, followed by primary aldosteronism at 53 years. At the age of 58, tolvaptan was started (60 mg/day) to treat her renal disease. After 8 months of tolvaptan therapy, hypokalemia-related muscle weakness became prominent, and hypertension became refractory. Finally, treatment with low-dose tolvaptan (30 mg/day) and high-dose spironolactone (100 mg/day) normalized serum potassium and the blood pressure. Tolvaptan can induce urinary excretion of potassium in patients with primary aldosteronism, and possible mechanisms are discussed.