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To be distinguished from aneurysm of an aortic sinus (Valsalva) is the rare condition in which an abnormal channel begins in the ascending aorta (above the right coronary artery), bypasses the aortic valve, and leads through the ventricular septum into the left ventricular cavity. This entity, which has not previously been diagnosed during life, to our knowledge, is herein named aortico-left ventricular tunnel. The cases of three patients (aged 3, 5, and 10 years) with this rare condition are described. The clinical, electrocardiographic, and radiological features were similar in each case and resembled those in aortic valvular insufficiency. The diagnosis may be established clinically through selective aortography. This reveals that the origin of the abnormal communication lies in the anterior wall of the ascending aorta above the levels of the coronary arterial origins. The abnormal communication leads to the left ventricle. The condition is distinguished from aortic sinus aneurysm by its origin above the level of the aortic sinuses. Identical anatomical findings and the presence of abnormal physical findings since infancy in each of the three patients suggest that this peculiar condition is a congenital malformation.