Abstract: Fibrosis is the characteristic hallmark of systemic sclerosis (SSc). Although the specific mechanism has been unknown, the role of macrophages in the development of this complex disease through various ways cannot be negligible. Findings during the past 5 years have contributed to more understanding of the relationship between macrophages and SSc. This review is mainly about the progress of research on macrophages in SSc. We discuss whether or not macrophages skew toward the M1-like phenotype from the profibrotic M2-like phenotype. Some biomarkers are also listed. In addition, we elaborate on the association of macrophages with other immune cells and Tall-like receptors.