AbstarctSpontaneous coronary artery dissection (SCAD) is a separation of the coronary wall layers, not related to trauma, medical procedures or atherosclerosis. The dissection causes the blood entry in the vascular wall with the consequent formation of a false lumen and intramural hematoma (IMH). Two pathogenetic mechanisms have been proposed to explain SCAD: a "primary" rupture of coronary endothelium or the rupture of the "vasa vasorum". Clinical presentation and severity of manifestations are variable, ranging from complete absence of symptoms to acute coronary syndrome (ACS), cardiogenic shock, cardiac arrest or sudden cardiac death. Despite coronary angiography is the first-line examination, by supplying two-dimensional images of the lumen, it does not always allow an incontrovertible diagnosis of SCAD. New intravascular imaging techniques, such as optical coherence tomography (OCT) and intravascular ultrasound (IVUS), have been recently introduced and may be extremely helpful in assessing the coronary wall integrity, thus improving coronary angiography diagnostic accuracy.Because of the lack of large randomized trials comparing different strategies, the optimal treatment of SCAD is still controversial. The first-line approach is conservative and based on medical therapy. Nevertheless, in particular situations an invasive approach is necessary. In the last years, several new strategies have improved the way to perform percutaneous coronary interventions (PCI), such as new generation drug eluting stents (DES), bio-resorbable scaffolds (BRS), sirolimus self-expandable stent (SES), drug eluting balloons (DEB), and cutting balloon. Cardiac artery bypass graft (CABG) is an even more invasive method to restore coronary flow and should be considered in urgent/emergent settings when PCI is not feasible or has failed.Cause the therapeutic approach of SCAD can be substantially different from that of atherosclerotic coronary artery disease, an accurate diagnosis is crucial to set up the best treatment strategy.