Japan has recently faced a public health crisis owing to “Beni Koji Choleste Help,” a product marketed as a dietary supplement. It is made from red yeast rice and has cholesterol-lowering effects. Following the identification of the first case of acute kidney injury (AKI) caused by the product, 76 possibly related deaths and 492 hospitalizations have been reported in Japan. We herein demonstrate 3 cases of AKI with renal tubular acidosis and Fanconi syndrome following consumption of the product for 2 weeks to 7 months.