A 13-year-old boy presented with an aggravated respiratory infection and orthopnea and was diagnosed with bicuspid aortic valve malformation, severe aortic valve insufficiency, and ascending aortic dilation. Despite in-hospital treatment with cardiac support, diuretics, and aggressive anti-infection therapy, the therapeutic effect was unsatisfactory. He later developed severe left heart failure, and the hemodynamics could not be maintained. He underwent an emergency Bentall procedure. The key points of anesthesia for such patients include avoiding drastic blood pressure fluctuations during surgery, minimizing aortic valve regurgitation to maintain cardiovascular homeostasis, and actively maintaining myocardial contractility postoperatively to balance myocardial oxygen supply and demand.