Fibromuscular dysplasia (FMD) is a segmental non-atherosclerotic, non-inflammatory vasculopathy typically of small- to medium-muscular arteries. Coronary FMD (CFMD) is believed to be rare. However, we have found an association between spontaneous coronary artery dissection (SCAD) and FMD as the cause of myocardial infarction in ∼25% of young women age <50 years. It is plausible that pre-existing CFMD predisposed these women to SCAD. Definitive diagnosis of CFMD entails autopsy that is obviously impractical, and prior angiographic description does not differentiate superimposed SCAD from obliterative arteriopathy of CFMD. Adjunctive intravascular ultrasound or optical coherence tomography may aid the diagnosis of CFMD and we report the first of such novel images. © 2012 Wiley Periodicals, Inc.