Aortico-left ventricular tunnel is a rare congenital communication between the ascending aorta and the left ventricle. Its hemodynamic effect is severe aortic incompetence. Surgery is the only treatment and should be performed before aortic incompetence or ventricular dilation develops. Two neonates with aortico-left ventricular tunnel were operated on at our institution, with closure of the aortic end of the tunnel with a Gore-Tex patch. The 2 patients were discharged in good conditions, and at 112 and 42-month follow-up respectively they are in good health, without medication and with a normal echocardiographic pattern. Aortico-left ventricular tunnel should be treated surgically as soon as possible in order to prevent any damage to the aortic valve and the left ventricle. The operative risk is not low, but results are very encouraging.