Coronary ectasia is defined as local or generalized aneurysmal dilatation of the coronary arteries. The present review summarizes the molecular, cellular and vascular mechanisms which are involved in the pathobiology of coronary ectasia. Coronary ectasia likely represents an exaggerated form of expansive vascular remodeling (i.e. excessive expansive remodeling) in response to atherosclerotic plaque growth. Enzymatic degradation of the extracellular matrix of the media is the major pathophysiologic process that leads to ectasia. Atherosclerotic lesions within ectatic regions of the coronary arteries appear to be highly inflamed high-risk plaques with proclivity to rupture. Better understanding of the pathogenetic processes involved in coronary ectasia is anticipated that will provide a further insight into the clinical significance and natural history of this entity, and may also have direct clinical implications in the management and follow-up strategy of this condition.