Aortico-left ventricular (Ao-LV) tunnel is a rare congenital anomaly that results in a communication between the ascending aorta and the LV. The clinical presentation is characterized by congestive heart failure and dilation of the ascending aorta within the first year of life due to significant regurgitation. There is considerable morphology variability which may affect the aortic valve and the right ventricular outflow tract. Prenatal diagnosis is not always contributory, and echocardiography remains the optimal imaging modality. Computed tomography is useful for surgical planning. Surgical intervention is usually indicated and typically involves patch aortoplasty. Following surgical repair, prognosis is good although long-term risk factors include progressive aortic regurgitation and ascending aortic dilatation.