Anomalies of origin, course, and distribution of coronary arteries, including single coronary artery, are well known in patients with Tetralogy of Fallot. However, to the best of our knowledge, there is no published case report of Pentalogy of Fallot with a single coronary artery. Herein, we introduce a 22-year-old female patient diagnosed via echocardiography and cardiac catheterization preoperatively as Pentalogy of Fallot with a single coronary artery arising from the left coronary sinus.