Tetralogy of Fallot (TOF) is the most common type of cyanotic congenital heart disease. The physiologic consequences that accompany the disease increase the risk for perioperative complications, and mortality is increased in the mere 3% that survive into adulthood without correction. This case report highlights the anesthetic management of a rare case of uncorrected TOF in a 50-year-old female with two-pillow orthopnea, occasional palpitations, and exertional dyspnea who underwent a major gynecologic procedure. Thorough understanding and careful planning were performed to balance anesthesia, accounting for shifts brought about by the long-term hypoxia and decreased pulmonary blood flow, affording an uneventful perioperative course and recovery.