A 39-year-old male with homozygous familial hypercholesterolemia confirmed by tissue culture suffered from mild aortic insufficiency and valvular stenosis with a gradient of 20 mm Hg across the aortic valve. Plasmapheresis carried out every 2 weeks for 4 years resulted in a marked reduction in the serum cholesterol level and in a regression of the valvular stenosis, as shown by echocardiography and by left heart catheter.