We reported a case of a 48-year-old male with homozygous familial hypercholesterolemia who underwent coronary revascularization successfully. Coronary artery bypass grafting (CABG) utilizing internal mammary artery graft to LAD, and percutaneous transluminal angioplasty for residual stenosis after CABG was able to relieve symptomatic myocardial ischemia. LDL apheresis every two weeks in addition to combined drug treatment had maintained total cholesterol at an acceptable level (120-280 mg/dl) before and after CABG. It was confirmed by repetition of coronary angiography at one year after CABG that all grafts were widely patent, and the native coronary artery did not accelerate the atherosclerotic lesion. It was important in patients with homozygous familial hypercholesterolemia, to carry out active coronary revascularization with reduction of serum cholesterol level by using LDL apheresis.