An abnormal communication between an artery of the systemic circulation and a pulmonary artery represents a rare clinical condition. A 76-year-old man presented with nocturnal dyspnea and retrosternal pain caused by a non-ST-elevation acute coronary syndrome. He had a history of a coronary artery bypass grafting operation 17 years previously, which required a repeat procedure 7 years later with a construction of an anastomosis of the left internal thoracic artery to the left anterior descending artery. Coronary angiography revealed an abnormal fistulous communication between the left axillary and the left pulmonary artery. Pressure measurements revealed pulmonary hypertension. An endovascular repair of the fistula was performed during repeat coronary angiography with an implantation of two self-expandable covered polytetrafluoroethylene stent grafts measuring 9 and 10 mm in diameter respectively, and thus resulting in a complete obstruction of the abnormal communication. During a follow-up period of 2 months the patient showed a significant improvement in his clinical condition.