Isolated coronary ostial stenosis is an unusual condition, most often atherosclerotic in origin. Only few cases have been reported as idiopathic. The clinical profile of the latter patients suggests that this group may represent a distinct clinical entity, with peculiar diagnostic and therapeutic features. We present our experience concerning six patients with isolated idiopathic coronary ostial stenosis, who successfully underwent transaortic left main ostioplasty with autologous pericardial patch fixed in glutaraldehyde. A review of previous reports in regard to the surgical management of coronary ostial disease is presented.