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Aorto-atrial fistulas (AAFs) are a relatively rare, but potentially life-threatening condition, where an anomalous connection forms between the aortic structures and the cardiac atria. AAFs are most often the result of an underlying condition concerning the cardiac structures. It may be congenital, secondary to conditions such as aortic dissection, infective endocarditis, or valve replacement, or iatrogenic in nature. Secondary causes incite local deterioration of cardiac wall integrity leading to formation of fistulous connections, whereas iatrogenic causes are more traumatic in nature. Signs and symptoms include those of volume overload and heart failure, with patients often presenting with fever, regurgitative murmurs, cardiac chamber dilation, and pedal edema. The diagnosis of AAFs requires a high degree of clinical suspicion necessitating imaging techniques such as echocardiography, computer tomography, and more invasive procedures. Management is dependent on underlying conditions and include the use of antibiotics, percutaneous closure, and in many cases, open heart surgery. It is important for physicians to be aware of this pathological condition to aid in timely management and favorable outcomes. This review attempts to summarize the various causes and clinical presentations of AAFs over the past decades.