A 76-year-old Japanese man with nephrotic syndrome was admitted to our department for treatment. After his admission, he was administered prednisolone (PSL) at 40 mg/day, and a percutaneous renal biopsy was performed. However, on the first day of admission, his urinary protein decreased from 5.05 g/gCr to 1.85 g/gCr. On the fourth day of admission, his urinary protein further decreased to 0.38 g/gCr and the patient developed acute kidney injury (AKI). Renin-angiotensin system (RAS) inhibitors were suspected to be the cause of AKI; therefore, they were discontinued. After the renal function improved, the urinary protein worsened again to 5.49 g/gCr. Renal pathology suggested minimal change disease (MCD); therefore, PSL was continued. The patient's urinary protein subsequently improved and he had no renal function impairment. Minimal change disease can be complicated by AKI through intravascular volume depletion caused by high urinary protein and hypoalbuminemia. However, when MCD is complicated by RAS inhibitor-associated AKI, the urinary protein may notably decrease, and the patient may present with an atypical course of MCDassociated AKI.