OBJECTIVES: Systemic sclerosis (SS) is a rare debilitating autoimmune disease of the connective tissue with no cure. This study aimed to assess the recent epidemiologic and economic burden of systematic sclerosis across the world. METHODS: Using a systematic search strategy, PubMed/MEDLINE and Embase were searched to identify relevant studies published from January 2006 to June 2016. Two independent reviewers evaluated studies for inclusion, quality, and extracted data. The review was conducted and reported according to PRISMA statement. RESULTS: CONCLUSIONS: This review provides an updated literature review on the current estimates of disease prevalence, incidence and economic burden of systemic sclerosis in different countries. The review reports slightly higher incidence/prevalence compared to previous studies. The economic burden is high from both societal and healthcare perspectives. Different healthcare stakeholders could benefit from understanding the epidemiological and economic aspects of SS.