Conventionally, mitral valve replacement (MVR) is performed under cardioplegic arrest. A 69-years-old male patient was admitted to our clinic with severe dyspnea, orthopnea and palpitation. Transthoracic echocardiography revealed fibro-calcified mitral valve stenosis. Without cross-clamping the aorta, beating heart MVR under cardiopulmonary bypass (CPB) was performed. Since no dissection of the ascending aorta is performed, the risks of injury and embolic complications are minimized. Porcelain aorta with the mitral valve disease is a new indication for beating heart MVR under CPB.