Dissection of ascending aorta is a medical emergency typically presenting with acute chest or back pain and hemodynamic instability. We are reporting a very unusual case of dissection of a large ascending aortic aneurysm presenting as a new onset heart failure. A 46-year-old man presented with gradually increasing dyspnea and orthopnea. His physical examination and laboratory findings were consistent with heart failure. The only unusual feature was a diastolic murmur, which prompted echocardiographic evaluation. Besides left ventricular dilatation, hypertrophy, and severe global hypokinesis, which were expected, we also found severely dilated aortic root with aortic regurgitation and a 8.6 × 9.7 cm ascending aortic aneurysm with dissection. The patient had a brother who died several years earlier from aortic dissection. Surgical treatment was successful. Type A aortic dissection may rarely present as heart failure. Aortic dissection at young age should prompt screening of first-degree relatives because genetic nature of the disease is very likely.