A fetus was diagnosed with regurgitation from the aorta into the left ventricle. A large aortico-left ventricular tunnel with aortic atresia was identified postnatally by echocardiography in a neonate suffering from severe heart failure. We successfully reconstructed the valve and closed the tunnel with a patch on his first day of life. At three-month follow-up, the baby is asymptomatic with mild aortic regurgitation. The combination of congenital aortico-left ventricular tunnel and aortic atresia can be misinterpreted in prenatal echocardiography as aortic regurgitation. This pathology may require urgent operative interventions.