This mini review evaluates mortality in SSc and provides a literature review concluding that premature death does occur in this population. However, there has been a changing spectrum of cause of death over the past three decades, with interstitial lung disease now being the commonest cause of SSc-related mortality. Cardiovascular (CV) mortality and events also contribute to the premature mortality seen in these patients, and this contention is supported by epidemiological studies, and further underpinned by a plethora of increased biomarkers for CV disease and events. Thus, macrovascular disease does occur in these patients, and is likely to contribute to mortality. It remains to be seen whether addressing conventional risk factors will attenuate CV disease in this population.