Left pulmonary artery agenesis, accompanied by a coronary arterial fistula, is a very rare anomaly. Although unilateral pulmonary artery agenesis is associated with other cardiovascular defects, like as ventricular septal defect, patent ductus arteriosus, and tetralogy of fallot, this anomaly, accompanied by a coronary arterial fistula, has not yet been reported. Most patients with no associated cardiac anomalies have only minor, or absent, symptoms, and survive to adulthood, but some patients may suffer from recurrent respiratory infections and hemoptysis. The vessel to the affected lung in many of the proved cases has been described as arising from either the bronchial artery or the aortic arch. The blood supply from the coronary artery to the affected lung has never been reported. Recently, a case of left pulmonary artery agenesis, accompanied with a coronary arterial fistula was experienced, which was diagnosed by coronary angiography and a chest CT, which is presented, with the review of relevant literature.