Aorta-right atrial tunnel is a rare and distinct congenital anomaly. To the spectrum of aorta-right atrial communications, we want to add this separate entity of aorta-right atrial tunnel by presenting the clinical features, morphologic aspects, diagnostic criteria, surgical techniques, and outcome in various clinical settings for better understanding of this developmentally intriguing, clinically complex, and therapeutically challenging disorder. From June 1994 through October 2004, 9 patients were treated for aorta-right atrial tunnel at our institution. Ages ranged from 9 to 45 years. There were 5 male subjects and 4 female subjects. Morphologically, 2 types of tunnels were identified in relationship to the ascending aorta: the anterior type in 3 patients and the posterior type in 6 patients. In all patients the diagnosis was established by means of 2-dimensional echocardiography and transesophageal echocardiography and confirmed by means of angiography. Eight patients were treated surgically, and 1 patient was treated with coil embolization. Postoperative echocardiograms obtained for all patients before discharge confirmed complete obliteration of the tunnel. One patient died perioperatively, and the other 8 patients were discharged in stable condition. During follow-up at 3 months, 6 months, and 1 year, all patients were in New York Heart Association class I, and echocardiography showed no residual shunts. In patients with aorta-right atrial tunnel, 2-dimensional echocardiography and transesophageal echocardiography are enough to establish clinical diagnosis, but ascending aortography is necessary to differentiate from more common clinical conditions, like ruptured sinus of Valsalva aneurysm and coronary cameral fistula. The rarity of this condition is established by the fact that during the same period of time, we have treated in our institution 66 cases of ruptured sinus of Valsalva aneurysm, which is the most common aorta-right atrial communication. Treatment options are simple ligation or ligation with implantation of coronary ostium or coil embolization. The location of the coronary ostium dictates technical details. Follow-up reveals excellent functional recovery.