Exosomes are small vesicles that can be secreted by most cells. Exosomes and their miRNA, mRNA and proteins play an important role in intercellular communication. Specifically, exosomes play an immunoregulatory role, participate in the pathogenesis and immunotherapy of autoimmune diseases, and can be used as effective biomarkers for diagnosis of diseases. In addition, exosomes can be used as biological carriers for drug delivery. Systemic sclerosis (SSc) is a complex autoimmune disease whose pathogenesis includes vascular damage, immune abnormalities, and fibrosis of internal organs and skin. In recent years, good progress has been made in the study of the pathogenesis of SSc, which has promoted research on clinical treatments. This article reviews the composition and function of exosomes, and their potential role in the pathogenesis, diagnosis and treatment of SSc.