Atherosclerosis (ATS) has been considered to be a degenerative disease affecting large and medium-sized arteries, resulting in a passive build-up of cholesterol in the artery wall. In the last decade, immune system was proved to play the key role in the pathogenesis of ATS, suggesting ATS to be more progressive and accelerated in chronic inflammatory conditions. Studies in patients with autoimmune diseases, particularly in the most prevalent ones such as rheumatoid arthritis and systemic lupus erythematosus, confirmed the significantly more serious atherosclerotic disease and increased cardiovascular (CV) risk compared to the general population, suggesting these diseases as an independent risk factor for CV diseases. There are only few studies evaluating ATS and CV risk in systemic sclerosis (SSc). Moreover, these studies present contradictory results. Furthermore, it is complicated to differentiate primary vascular affection related to the pathogenesis of SSc from the secondary vascular infliction due to ATS. Nevertheless, most of the studies to date suggest ATS and its clinical manifestations to be more prevalent in SSc. Future studies evaluating larger cohorts of patients are required to determine the relevance of ATS and CV disease and management of these comorbidities in SSc.