Systemic sclerosis is a highly morbid, complex autoimmune disease that is variable both in its phenotype and the attendant mortality driven by such manifestations. This review article synthesizes mortality data from the best available meta-analyses, subgroup analyses of single cohorts, and subjective comparisons of individual cohort studies, which in aggregate suggest that mortality in systemic sclerosis has been gradually improving over the past several decades. This review also summarizes the literature describing various risk factors for mortality in systemic sclerosis.