Coronary artery ectasia (CAE) is a rare but clinically important vascular abnormality defined by localized or diffuse dilation of a coronary artery segment to at least 1.5 times the diameter of adjacent normal vessels. The prevalence and clinical impact of CAE vary widely by geography and ethnicity, with higher rates seen in Mediterranean, Middle Eastern, and Asian populations. While over half of cases are associated with atherosclerosis, congenital anomalies, connective tissue disorders, vasculitis, and trauma represent important non-atherosclerotic etiologies. Recent insights into chronic inflammation, extracellular matrix degradation, genetic predisposition, and epigenetic alterations have expanded the understanding of CAE pathogenesis. Although many patients remain asymptomatic, CAE can present with angina, myocardial infarction, or even sudden cardiac death. Diagnostic advances, such as coronary computed tomography angiography and intravascular ultrasound, enable more precise evaluation, but standardized criteria and management guidelines remain lacking. Current management focuses on risk factor modification and antithrombotic therapy, though the optimal regimen is uncertain, and the efficacy of invasive interventions is not well established. Long-term prognosis is highly variable, underscoring the need for region-specific studies, robust clinical trials, and the development of personalized care strategies. Future research priorities include genetic and molecular studies, creation of evidence-based protocols, and the exploration of novel therapeutics to improve outcomes for patients with CAE.