Elevated risk of cardiovascular disease (CVD) in chronic kidney disease (CKD) is attributable to traditional and nontraditional risk factors. Recent studies showed that patients with reduced renal function have increased cholesterol absorption and decreased cholesterol synthesis, and that the serum markers of cholesterol metabolism predict all-cause death in hemodialysis patients. So far, their relationships with CVD events have not been examined in this population.We tested a hypothesis that the altered cholesterol metabolism is predictive of CVD events in hemodialysis patients. Prospective cohort study in 492 patients undergoing maintenance hemodialysis without treatment with statin or ezetimibe in Japan. The key exposures were tertiles of serum lathosterol (as a marker of cholesterol synthesis) and serum campesterol (as markers of cholesterol absorption). The main outcome was composite CVD events including coronary, cerebral, and peripheral arterial events pulse congestive heart failure and valve disease during 5-year follow-up. At baseline, hemodialysis patients had lower lathosterol and higher campesterol concentrations as compared with the 100 healthy controls. During follow-up, we documented 181 CVD events and 102 all-cause deaths. When adjusted for age, sex, dialysis duration, diabetes mellitus, and prior CVD history, a lower lathosterol level was associated with CVD events, but campesterol was not. A lower lathosterol level was significantly associated with all-cause death, whereas campesterol showed a U-shaped association with all-cause death in an adjusted model. A higher campesterol to lathosterol ratio was associated with higher risks of both CVD event and all-cause mortality. In hemodialysis patients, serum lathosterol was lower and campesterol was higher than control levels. The results of this study suggest that a lower cholesterol synthesis, rather than increased cholesterol absorption, was predictive of future CVD events.