The existence of myocardial infarction despite angiographically normal coronary arteries was recognized more than 30 years ago. Since then, various series of such patients have been described, but the aetiology and pathogenesis of the condition are still a source of debate. Evidence exists for a role of coronary vasospasm, thrombosis, embolization and inflammation, per se or combined, in determining the occurrence of myocardial infarction in the presence of angiographically normal coronary arteries. Endothelial dysfunction, possibly superimposed to nonangiographically evident atherosclerosis, may be an underlying common feature predisposing to the acute event. Additionally, myocarditis may explain some of these occurrences. Myocardial infarction with normal coronary arteries is therefore likely the result of multiple pathogenetic mechanisms. Although most reports emphasize the good prognosis of this condition, in general much better than myocardial infarction with coronary artery disease, prognosis is likely variable according to the underlying mechanism. This review summarizes current knowledge on this condition and examines areas of recent progress.