Systemic sclerosis (SSc) is a chronic disease of unknown etiology, characterized by enhanced fibrosis, and microvascular abnormalities. During the past several decades, the death rates due to cardiovascular disease or cerebrovascular disease in SSc patients substantially increased and are currently responsible for 20-30% of mortality. Various autoimmune rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus accelerate atherosclerosis. Although microvascular disease is a hallmark of SSc, an ongoing debate exists regarding the presence and extent of macrovascular diseases and the presence of accelerated atherosclerosis in SSc patients. Despite conflicting results as to intima-media thickness (IMT) in SSc patients, the most recent and largest study has found no difference in either plaque occurrence or IMT. Additionally, abnormal coronary flow reserve in SSc patients appears to be due to microvascular involvement rather than atherosclerosis of the epicardial coronary arteries. Angiographic findings as well as computed tomography studies have generated conflicting reports as to coronary atherosclerosis in SSc. Herein, we review the current knowledge of macrovascular involvement and atherosclerosis in SSc. The differences between SSc and other autoimmune rheumatic diseases in the presence and extent of atherosclerosis need to be further investigated.