We report on a 22-year-old female with focal glomerular sclerosis and hyperlipidemia who did not respond to long-term steroid or immunosuppressant therapy. When low-density lipoprotein (LDL) apheresis was performed, her total daily protein excretion decreased, serum albumin increased, total cholesterol decreased from 1,052 mg/dl to 148 mg/dl after 3 months, and the serum lipoprotein(a) level also decreased from 117.8 mg/dl to 9.1 mg/dl. After this therapy, her clinical course was well maintained. By controlling hyperlipidemia, including oxidized low-density lipoprotein and lipoprotein(a), low-density lipoprotein apheresis may produce clinical improvement in focal glomerular sclerosis.