A 41-year-old woman with recent onset of heart failure and angina due to aortic valve incompetence and critical left coronary ostium stenosis in the setting of Takayasu's arteritis is reported. The patient was successfully surgically treated by aortic valve replacement and coronary artery bypass with saphenous vein graft, showing a cardiac event-free 17 months follow-up. Takayasu's arteritis must be included among the possible causes of coronary artery disease and aortic valve incompetence in young female patients. Although chronic inflammation of the aortic wall may result in late graft occlusion, surgical therapy is effective for short and mid-term clinical improvement.