Background. Although hemodialysis (HD) patients have been associated with elevations in serum lipoprotein(a) [Lp(a)] levels, relatively little has been published on the link between Lp(a) and the risk for atherosclerotic cardiovascular death in HD patients. Methods. Lipoprotein(a) was measured in 390 HD patients. The relationship between Lp(a) and mortality (overall and cardiovascular) was determined during 28 months of prospective follow-up. Results. Hemodialysis patients demonstrated Lp(a) concentrations that were approximately two times as high as that of healthy controls (median, 16 vs. 8 mg/dl, P < 0.001; mean, 22.9 vs. 12.1 mg/dl, P < 0.01). Lp(a) showed a significant correlation between albumin, total cholesterol, low-density lipoprotein cholesterol, and C-reactive protein. The high-Lp(a) group [Lp(a) greater than or equal to 30 mg/dl] showed significantly higher mortality than the low-Lp(a) group [Lp(a) < 30 mg/dl] in a Kaplan-Meier survival analysis (P < 0.05). Multiple logistic regression analysis demonstrated albumin, age, and diabetic state as significant risk factors for overall death. However, if confined to atherosclerotic cardiovascular death, Lp(a) (P < 0.01), age, and diabetic state were the only independent contributors. Conclusions. Lp(a) is an independent risk factor for atherosclerotic cardiovascular death in Japanese patients receiving chronic dialysis therapy.