Thoracic aortic aneurysm and dissection (TAAD) is a life-threatening cardiovascular disorder lacking effective clinical pharmacological therapies. The underlying molecular mechanisms of TAAD still remain elusive with participation of versatile cell types and components including endothelial cells (ECs), smooth muscle cells (SMCs), fibroblasts, immune cells, and the extracellular matrix (ECM). The main pathological features of TAAD include SMC dysfunction, phenotypic switching, and ECM degradation, which is closely associated with inflammation and immune cell infiltration. Among various types of immune cells, macrophages are a distinct participator in the formation and progression of TAAD. In this review, we first highlight the important role of inflammation and immune cell infiltration in TAAD. Furthermore, we discuss the role of macrophages in TAAD from the aspects of macrophage origination, classification, and functions. On the basis of experimental and clinical studies, we summarize key regulators of macrophages in TAAD. Finally, we review how targeting macrophages can reduce TAAD in murine models. A better understanding of the molecular and cellular mechanisms of TAAD may provide novel insights into preventing and treating the condition.