The CCN family comprise the products of six immediate-early response genes (Cyr61, Ctgf, Nov and Wisp1-3) and are multi-functional proteins, characterised by four discrete protein modules in which reside functional domains: an insulin-like growth factor binding protein-like module (IGFBP) but has low affinity for IGFBPs, a von Willebrand factor type C repeat module (VWC) which mediates integrin and growth factor binding, a thrombospondin type-1 repeat module (TSP-1), and a cysteine-knot-containing module (CT). These modules mediate a host of interactions such as growth factor binding, integrin recognition, and interaction(s) with heparin and proteoglycans (reviewed in Holbourn et al., 2008; Chen and Lau, 2009). The CCN family are involved in many normal and pathological cellular processes and have a plethora of functions including cell proliferation, angiogenesis, wound healing, and fibrogenesis, tumourigenesis. In addition, many roles have been described for CCN family members in the cardiovascular system (Table 1). The focus of this review is the role of connective tissue growth factor (CCN2, CTGF) in blood vessels and in vascular pathology.