Fibromuscular dysplasia (FMD) of the epicardial coronary arteries is an uncommon entity. We describe the first published case of this condition presenting as a transient ischaemic attack due to thromboembolism from intracardiac thrombus secondary to silent myocardial infarction. We also present the first published case of epicardial coronary FMD with sparing of the renal arteries. This case provides further evidence that FMD of the epicardial coronary arteries frequently involves the mid to distal segments of the left anterior descending artery. A brief review of the literature on FMD is presented.