Cholesterol embolic disease is a devastating complication of atherosclerosis. Universally recommended treatment is lacking thus far. Recent data suggest that a therapeutic protocol aimed at specifically combating three causes of mortality, recurrent bouts of cholesterol embolism, cardiac failure, and cahexia, were associated with a favorable clinical outcome. As for drug therapy, corticosteroid has been reported to be beneficial in reducing local and general inflammatory responses. Concerning apheresis, combined therapy consisting of plasma exchange and low to intermediate-dose corticosteroid therapy has been shown to be effective in multivisceral cholesterol embolism. Low density lipoprotein (LDL) apheresis has been reported to be beneficial for cholesterol embolism-induced damage to the skin and brain.