The coronary artery-left ventricular microfistula is an extremely rare congenital anomaly. Little is known about their epidemiologic and clinical features. Moreover, to our knowledge, only two cases of coronary artery-left ventricular microfistulae demonstrated by transthoracic doppler echocardiography have been reported in the literature. Recently we exerienced a case of coronary artery-left ventricular microfistulae demonstrated by transthoracic doppler echocardiography, who was a 63 year old woman and visited for evaluation of chest pain. Herein, along with a review of the pertinent literature regarding this disorder, we report a case of coronary artery-left ventricular microfistulae demonstrated by transthoracic doppler echocardiography.