We sought to assess the angiographic and long-term clinical outcomes in a predominantly medically treated population with spontaneous coronary artery dissection (SCAD). There are little data on the angiographic and long-term outcome in patients with SCAD. We studied 64 patients with SCAD (mean age 53 years, 94% females, three peripartum) with acute coronary syndrome who were treated using coronary bypass grafting (n = 1), percutaneous coronary intervention (n = 7), or medical therapy (n = 56). A repeat angiogram was performed in 40/64 (63%) patients. The median clinical follow-up was 4.5 years. Five (8%) patients had a major cardiac event. One patient with peripartum left main SCAD and cardiogenic shock died during PCI. One patient with conservatively treated SCAD of the posterior descending artery suffered out-of-hospital cardiac arrest 16 days after the initial angiogram but survived. Three patients experienced a second SCAD in another vessel 3.7, 4.7, and 7.9 years after the index event while the initial dissection had healed. Thirty medically treated patients underwent a scheduled repeat angiogram showing healing of the dissection in all but one patient. After a median follow-up of 4.5 (1.8-8.4) years, all 63 patients surviving the index event were alive and free of symptoms suggestive of myocardial ischemia. In general, the long-term outcome of patients with SCAD is excellent, and medical therapy can be safely applied in the majority of patients. However, SCAD can be a life-threatening and sometimes catastrophic event, and some patients experience early or late complications including SCAD of another vessel. © 2015 Wiley Periodicals, Inc.