Lipoprotein(a) (Lp[a]) is a LDL-like particle consisting of an ApoA moiety linked to one molecule of ApoB(100). Recent data from large-scale prospective studies and genetic association studies provide highly suggestive evidence for a potentially causal role of Lp(a) in affecting risk of cardiovascular disease (CVD) in general populations. Patients with Type 2 diabetes display clustered metabolic abnormalities and elevated risk of CVD. Lower plasma Lp(a) levels were observed in diabetic patients in several recent studies. Epidemiology studies of Lp(a) and CVD risk in diabetic patients generated inconsistent results. We recently found that Lp(a)-related genetic markers did not predict CVD in two diabetic cohorts. The current data suggest that Lp(a) may differentially affect cardiovascular risk in diabetic patients and in the general population. More prospective studies, Mendelian randomization analysis and functional studies are needed to clarify the causal relationship of Lp(a) and CVD in diabetic patients.