Abstract: Coronary artery ectasia (CAE) is a vascular lesion characterized by abnormal dilation of the coronary arteries, with complex pathogenesis and diverse clinical manifestations. CAE can be classified into primary and secondary types. This article aims to systematically review the epidemiology, definition, core pathophysiological mechanisms, and clinical management strategies of primary CAE. It focuses on exploring the central roles and interactions of lipid metabolism disorders and chronic immune-inflammatory responses in the development and progression of the disease. Additionally, current therapeutic challenges and future research directions are discussed, with the goal of providing reference for clinical practice and basic research.