Tetralogy of Fallot (TOF) is a cyanotic congenital heart defect comprising stenosis or atresia of the pulmonary valve, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. TOF is known to be associated with coronary artery anomalies. We describe a rare case of TOF associated with an intracavitary right coronary artery (RCA) course. The recognition of such an anomaly is vital before surgical/interventional procedures to avoid catastrophic outcomes.