Tetralogy of Fallot is a common cyanotic congenital heart disease which is often treated surgically in infants and young children. Due to long-term hypoxemia and cardiopulmonary dysfunction, adult patients with tetralogy of Fallot usually have poor tolerance to anesthesia and surgery, which brings great challenges to perioperative management. Recently, our hospital successfully completed anesthesia and perioperative management of thoracic spinal canal tumor resection in a 50-year-old female uncorrected tetralogy of Fallot in prone position. Strategies of anesthesia management were applied, including invasive blood pressure monitoring before anesthesia induction, induction with appropriate doses of intravenous agents at an appropriate rate, and precise anesthesia management through the surgery. Based on our cooperation between anesthesia, intensive care, and surgical team, we completed the operation successfully and the patient was discharged from our hospital 10 d after surgery.