Spontaneous coronary artery dissection (SCAD) is an infrequent condition that is underdiagnosed. There is a predilection for young women without traditional cardiovascular risk factors, and it is increasingly diagnosed in women who are not peripartum. We discovered an association between SCAD and fibromuscular dysplasia (FMD), in which most women with nonatherosclerotic SCAD were found to have FMD in another vascular territory. We suspect that these seemingly healthy patients have underlying coronary FMD that predisposed them to coronary dissection. Medical treatment of SCAD includes antiplatelet therapy and β-blockade. Revascularization of SCAD patients might be challenging, and the recommendation for stenting or surgery depends on their clinical status and the dissected coronary anatomy. The long-term outcome of patients who survived their SCAD event is generally good, however, they are at risk for recurrent dissection and major cardiovascular events, and thus should be closely monitored by cardiovascular specialists. This review summarizes the epidemiology, associated etiology, diagnosis, management, and outcome of patients with SCAD.