Atherosclerosis is considered to be a chronic inflammatory disorder. Several autoimmune rheumatic diseases are characterized by premature and accelerated atherosclerosis in which both classical and non-classical risk factors contribute to atherogenesis. SSc is characterized by vasculopathy, and microvascular involvement is common. Macrovascular involvement is considered rare, although increased prevalence of macrovascular disease has been reported as well. Here, we review the literature regarding coronary artery disease, cerebrovascular disease and peripheral arterial disease in SSc. An increased prevalence of distal peripheral artery disease in the digits has been found. The prevalence of coronary artery disease and cerebrovascular disease is not increased, although studies using intima-media thickness of the carotid artery as a marker of early atherosclerosis showed discrepant results. Besides traditional risk factors, as present in the general population, non-traditional risk factors are present in SSc as well, such as increased lipoprotein(a), oxidized LDL, inflammation, vasospasm and endothelial dysfunction. Moreover, markers of vascular damage in atherosclerosis, like antibodies to oxidized LDL, and increased levels of soluble vascular adhesion molecules, have been described in association with vascular damage in SSc. Nevertheless, generalized premature atherosclerosis has not been detected in SSc. Therefore, further research is necessary to assess the prevalence of clinically manifest or subclinical early atherosclerosis in SSc.