An 11-year-old girl experienced several syncopal attacks and her electrocardiogram showed ST depression with exercise. Ostial stenosis of the left coronary artery was disclosed by the coronary angiography. We suspected the coronary involvement was due to Takayasu's arteritis. On operation, aortitis in the ascending aorta involving ostium of the left coronary artery was observed. Therefore Takayasu's arteritis is considered to be one of the causes of sudden death.