An 11-year-old girl with Takayasu arteritis was presented with recurrent chest pain. A transthoracic echocardiogram showed moderate to severe aortic regurgitation with impaired cardiac function. Cardiac catheterization revealed the total occlusion of the small right coronary artery and left main stenosis of 40%. An intraoperative transesophageal echocardiogram showed a redundant intimal fold partially covering the left coronary ostium and this was further confirmed from the intraoperative finding. This is a case report of recurrent angina associated with obstruction of left main coronary artery orifice by an intimal fold secondary to Takayasu arteritis.