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Tolvaptan (TLV) is a vasopressin V2 receptor antagonist that increases free water excretion. However, there are few reports of the use of TLV in pediatric patients with nephrotic syndrome (NS). The efficacy of TLV for the management of edema and hyponatremia in a 12-year-old boy with refractory NS is demonstrated. In this patient, refractory NS developed at 5 years of age, and remission was maintained with several immunosuppressive agents. He was admitted to hospital due to relapse with oliguria, edema, hypoalbuminemia, and hyponatremia