Sinus of Valsalva aneurysms are rare cardiac anomalies which may be acquired or congenital, most commonly involving the right or noncoronary sinuses. The congenital aneurysms are more common and often caused by weakness at the junction of the aortic media and the annulus fibrosus. Acquired aneurysms are caused by conditions affecting the aortic wall, such as infections (syphilis, bacterial endocarditis, or tuberculosis), trauma, or connective tissue disorders. Unruptured aneurysms are usually found incidentally during diagnostic studies. More commonly, sinus of Valsalvaaneurysms are diagnosed after clinical sequelae of rupture. Diagnosis of sinus of Valsalva aneurysm is facilitated by echocardiography, contrast aortography, and more recently, magnetic resonance imaging. Repair is generally required for ruptured aneurysms; unruptured aneurysms encroaching on nearby structures, causing myocardial ischemia, or having the potential to rupture warrant repair. A review of the literature is presented focusing on anatomy, clinical presentation of ruptured and unruptured aneurysms, noninvasive diagnostic modalities, and techniques for repair of this anomaly.