Aortic dissection (AD) is a life-threatening condition and may present with symptoms which mimic myocardial infarction, leading to misdiagnosis and inappropriate use of anticoagulant and thrombolytic therapy. A 40-year-old woman with no prior history presented in our emergency department with sudden chest pain. Electrocardiography (ECG) showed a ST-segment elevation in leads II, III and avF, suggesting an acute inferior myocardial infarction. The patient was given anticoagulation and antiplatelet treatment. Coronary angiography, transthoracic echocardiography and computed tomography were performed. The patient was diagnosed with DeBakey I aortic dissection extending from ascending aorta to iliac artery, and associated with bicuspid aortic valve.Surgical treatments with a replacement of the ascending aorta, aortic valve replacement and coronary artery bypass grafting were successfully performed. Early imaging examination, if possible, might assist the diagnosis and guide the management of this disease. The condition of myocardial infarction secondary to aortic dissection is discussed.