Unroofed coronary sinus with persistent left superior vena cava is an extremely rare congenital anomaly. It can be suspected by the presence of a markedly dilated coronary sinus and can be confirmed by injection of agitated saline into the left antecubital vein during echocardiography. Agitated saline contrast echocardiography can easily visualize the flow in the persistent left superior vena cava communicating with the unroofed coronary sinus hereby creating a shunt into the left atrium. An agitated saline contrast echocardiography may be performed with a low risk of transient complication. We demonstrate the case of a 32-year-old pregnant woman with unroofed coronary sinus and persistent left superior vena cava, confirmed by agitated saline contrast echocardiography.