We report the case of a patient with mitral regurgitation complicated by type B dissection and Marfan syndrome (MFS) who was managed successfully with minimally invasive mitral valve repair. Without type A aortic dissection or aortic root dilation, MFS patients may develop mitral valve regurgitation, as in this case, and need valve surgery to improve their symptoms and long-term survival. However, it is not clear that a full sternotomy and prophylactic aortic surgery are necessary. Although retrograde perfusion to the dissected aorta is controversial, our approach minimizes the risk of future anticipated aortic surgery in MFS patients.