Background and Aim: Conventional mitral valve replacement (MVR) is carried out under cardioplegic arrest with cross-clamping of the ascending aorta during cardiopulmonary bypass. In this case, MVR was performed with on-pump beating heart technique without cross-clamping the aorta because of the diffuse adhesion around the ascending aorta, and tube graft presence between ascending and descending aortas. Methods: A 47-year-old female patient had aorto-aortic bypass graft from ascending aorta to descending aorta with median sternotomy and left thoracotomy in single stage because of aortic coarctation 2 years ago in our cardiac center. She was admitted to the hospital with palpitation and dyspnea on mild exertion. Transthoracic echocardiography revealed 4th degree mitral insufficiency. Results: MVR was carried out through remedian sternotomy with on-pump beating heart technique without cross-clamping the aorta. Conclusions: MVR with on-pump beating heart technique offers a safe approach when excessive dissection is required to place cross-clamp on the ascending aorta.