Spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic coronary artery pathology and an important cause of coronary artery disease in young women with an average age of 40 to 50 years with few or no cardiovascular risk factors. There has been a surge in the diagnosis of SCAD due to an increased use of coronary angiography and the clinical availability and application of high-resolution intracoronary imaging. SCAD is due to the separation of coronary wall layers with the formation of intramural hematoma, compression of the true lumen and secondary myocardial ischemia. Coronary angiography is the first-line imaging, also useful are intravascular ultrasound and optical coherence tomography. Therapy is conservative in most cases because of the high percentage of spontaneous healing of the vascular wall. The prognosis is good, although the disease is burdened by a high prevalence of major adverse coronary events, including recurrence of coronary dissection, thus making careful follow-up essential in survivors of the acute event. Given that the presence of SCAD may be associated with aneurysm formation and dissections of other arteries, screening of the arterial tree may be useful, especially of the supra-aortic trunks and splanchnic circulation.