LDL-apheresis is now commonly used as the only practical treatment for homozygous familial hypercholestreolemia (homozygous FH). However, even when applying apheresis therapy, the use of a drug or drugs is recommended to suppress the rapid rebound of cholesterol, which usually takes place after each apheresis procedure, and keep the LDL-cholesterol level within or near the optimal range for as long as possible. In this study, the usefulness of ezetimibe, a novel cholesterol-lowering drug, in enhancing the efficacy of apheresis therapy was evaluated in six Japanese patients with homozygous FH undergoing LDL-apheresis in combination with atorvastatin or simvastatin. With the exception of one patient, significant decreases in LDL-cholesterol at 2 weeks after each apheresis procedure were obtained during the period from 4 to 12 weeks of treatment, with an average reduction rate of 9.0% and a range of 4.3-12.6%. This corresponds to a suppression of rebound by approximately 36 mg/dl, from 391 to 355 mg/dl on average, in LDL-cholesterol values. Although the effect is not very strong, ezetimibe nevertheless appears to be a useful drug in combination with statins for those with homozygous FH undergoing LDL-apheresis.