Intracranial arterial dolichoectasia (IADE) is a major arterial disease characterized by artery elongation, dilatation, and curvature. Although the pathological mechanism of IADE is unclear, recent studies have suggested a close relationship with matrix metalloproteinases (MMPs). This review summarizes the possible pathological mechanism of IADE induced by MMPs in terms of MMPs activation and the occurrence and development of IADE, the correlation between MMPs activation and positive remodeling causing IADE, and the relationship between hemodynamic changes, MMPs activation and IADE and between IADE-associated diseases and MMPs, to provide potential therapeutic targets for the effective prevention and treatment of IADE.