There is an increased risk of cardiovascular disease (CVD) mortality and morbidity in patients with type 1 diabetes mellitus compared with the general population as shown by epidemiologic studies measuring cardiovascular endpoints, as well as by autopsy, angiographic, and coronary calcification studies. Most of the excess CVD risk associated with type 1 diabetes is concentrated in the subset of approximately 35% of patients who develop diabetic nephropathy (after 20 years of diabetes duration), who also typically have dyslipidemias, elevated blood pressure, and hyperglycemia, factors contributing to CVD. For reasons that remain speculative, the relative risks from CVD are higher in women than in men with type 1 diabetes compared with the general population, which effectively eliminates the gender differences in CVD. As in the general population and in patients with type 2 diabetes, education and lifestyle changes, interventions to reduce hyperglycemia, blood pressure, micro-albuminuria, lipid control, and the use of aspirin are important management areas in order to reduce the increased risk of CVD. Whether management with aspirin and statins should be started in type 1 diabetic patients at a younger age or at a lower risk score than in the general population is still under investigation. There is a need for a better understanding of the pathophysiology of vascular complications in type 1 diabetes, more specific risk engines in type 1 diabetes, and accurate estimations of the absolute and relative risk for CVD in order to improve management of CVD in these high-risk patients.