A 28-day-old male neonate presented with cyanosis, skin rash, and lethargy. Echocardiography indicated “congenital aortic valve stenosis.” After endotracheal intubation, mechanical ventilation support, and appropriate medical therapy, an emergent aortic valve commissurotomy was scheduled under general anesthesia. Aortic valve stenosis not only causes the increase of the left ventricular afterload and myocardial oxygen demand but also reduces the forward blood flow and results in inadequate perfusion of the systemic and coronary circulations. The survival of neonates with severe aortic valve stenosis hinges upon the patency of the arterial duct and the pulmonary to aorta artery shunting, which results in differential cyanosis, metabolic acidosis, and myocardial ischemia. In this case, the key to anesthetic management is to maintain the patency of the arterial duct, optimize the balance between myocardial oxygen supply and demand, and preserve myocardial function.