Introduction: Spontaneous coronary artery dissection (SCAD) represents a very rare and poorly understood condition that is gaining recognition as an important cause of myocardial infarction, especially among young women. The pathogenesis of SCAD is not well established yet, but several theories have been proposed. Case presentation: We report the case of a 25-year-old woman without any history of cardiovascular disease who presented with acute anterior ST-elevation myocardial infarction (STEMI) due to the luminal obstruction generated by an intramural hematoma from a SCAD of the left main coronary artery, which was successfully treated by coronary artery stenting. Additionally, the patient presented anomalies of coronary origins (ACO) with separate emergences of the left anterior descending (LAD) artery from the left coronary cusp and the left circumflex artery (LCX) from the right coronary cusp, with no apparent clinical significance. Conclusion: SCAD should always be included in the differential diagnosis of young patients presenting with STEMI. In case of prompt diagnosis, SCAD-STEMI patients are successfully treated with percutaneous coronary intervention (PCI). Moreover, it is of vital importance to identify variants of ACO, even without clinical relevance at the moment of the acute event, in order to initiate an appropriate management, since ACO increases the risk of routine PCI.