Aneurysm of the sinus of Valsalva is a rare cardiac anomaly due to the nonspecific symptoms and dif-ficult confirmation of diagnosis leading to high mortality. This report describes the case of a 53-year-old man who had suffered from acute chest pain. The electrocardiogram showed the ST segment ele-vation in lead aVR and ST-T wave changes in leads I, II and V4-6. Elevated D-dimer levels and cardi-ac enzymes were also observed. Acute myocardial infarction caused by the left main coronary artery obstruction was initial diagnosed. Coronary angiogram was performed immediately and the result indicated non-obstructive coronary artery. However, cardiogenic shock with persistent chest pain was discovered. Aortic dissection, pulmonary thromboembolism and cardiac tamponade were gradually excluded. Through detailed review and analysis of medical history, physical assessment and image ex-aminations with echocardiography, chest computed tomography and coronary angiogram, a ruptured sinus of Valsalva aneurysm was eventually diagnosed based on image examinations which revealed left-to-right shunt communicating with the right-sided heart chambers. The patient received emergen-cy right coronary sinus aneurysm repairment and aortic valve replacement surgery. Symptoms with chest pain and shock gradually disappeared and left ventricular function was restored postoperatively. The patient demonstrated excellent recovery. In facing relevance diagnostics for patients with acute chest pain and hemodynamic instability, a ruptured sinus of Valsalva aneurysm must be considered to endure best treatment timing if heart auscultation reveals S1 and S2 continuous murmurs.