Persistent left superior vena cava is a rare congenital abnormality. Cases of persistent left superior vena cava with an absent right superior vena cava or the presence of other congenital cardiovascular abnormalities have been rarely described. To the best of our knowledge, this is the first case of a patient with persistent left superior vena cava, absent right superior vena cava and tako-tsubo syndrome observed during an attempt of pacemaker implantation. Such a condition was confirmed by means of contrast echocardiography and coronary angiography.