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Acute coronary artery dissection is a rare, complex disease occurring particularly in young women without traditional cardiovascular risk factors. The pathophysiology and treatment are different from acute coronary syndrome caused by plaque rupture or erosion. Its clinical presentation may vary from unstable angina to sudden cardiac death. Hence, early detection is crucial to manage the dissection and reduce the mortality and morbidity rates. Most coronary dissections will heal spontaneously, and conservative treatment is recommended for uncomplicated cases. In the acute phase, primary percutaneous coronary intervention remains the reperfusion strategy of choice; however, in small- and medium-sized arteries with normalized blood flow, conservative treatment is beneficial. Medical therapy should be tailored to the individual depending on the underlying severity of the condition. Percutaneous coronary intervention should be performed by experienced operators, with the use of intravascular imaging, and preferably with on-site surgical back-up due to the increased risk of complications. The prognosis is favorable; however, patients have a high risk of recurrent dissections in other arteries several weeks after the first event, suggesting a general weakness of the arteries. In the recent past, optical coherence tomography has played an important role in the diagnosis of acute coronary artery dissection; however, its therapeutic potential is underestimated. We recommend that long-term clinical trials should be conducted to fully determine the long-term mortality and morbidity rates of these patients.