Low-density lipoprotein apheresis (LDL-A) has been developed as a therapy for familial hypercholesterolemia, but LDL-A has also been used as a general treatment for drug-resistant nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS). The patients with NS due to minimal change disease (MCD) are often difficult to control effective circulating plasma volume, causes acute kidney injury (AKI), and when diuretics are not effective and the respiratory condition of patients worsens, patients require acute renal replacement therapy (ARRT). The effectiveness of LDL-A is not only reduction of serum low-density lipoprotein but also various other benefits. LDL-A might have improved renal hemodynamics by reducing vasoconstrictive eicosanoids and contributed to the therapeutic effect of antiproteinuric drugs such as corticosteroids. We treated a 49-year-old Japanese woman and a 71-year-old Japanese man with AKI caused by NS due to MCD, who required ARRT. Although these patients received ARRT and corticosteroids, their AKI and MCD did not improve sufficiently. We initiated LDL-A treatment for these patients as an additional treatment modality, because their total serum cholesterol levels were high at the time of admission. After the additional LDL-A treatment, both patients were able to discontinue ARRT, because NS and AKI in both patients were improved sufficiently. It is possible that early additional LDL-A is effective for patients with AKI and NS due to MCD who require ARRT, and may help patients discontinue ARRT because of the effect of LDL-A such as improving hypercoagulability and renal hemodynamics and contributing to the therapeutic effect of corticosteroids.