Clustering of metabolic syndrome (MetS) components within individuals helps in the identification of subjects who are at increased risk for both cardiovascular disease (CVD) and diabetes. In this review we describe how the presence of MetS influences CVD risk. Our review focuses on published studies through May 2006 referring to incident CVD in relation to the National Cholesterol Education Program Adult Treatment Panel III (NCEP) definition of the MetS. We present data suggesting that the Framingham risk function is the most appropriate method for assessing CVD risk in subjects with or without MetS. We show how the CVD risk associated with MetS is influenced by the inclusion of subjects with diabetes and CVD at baseline, and by the development of diabetes during follow-up, and that this might explain why MetS may be a stronger risk factor for CVD in women than in men. We present data suggesting that CVD risk associated with MetS does not appear to be greater than the sum of its parts, and that adding CRP to MetS variables does not improve population CVD risk prediction. Lifestyle intervention and treatment of specific abnormal MetS components are appropriate until a better understanding of the pathogenesis of MetS is available. At such time we may be able to target the underlying causes of the syndrome and ultimately prevent the development of both CVD and diabetes.