Patients with Marfan syndrome often have severe life-threatening cardiovascular complications, including aortic aneurysmal dilatation or aortic dissection. Approximately two-thirds of people who have this syndrome suffer from chest wall deformities such as pectus excavatum (cobbler's chest) or pectus carinatum (bird's chest). When a patient with cobbler's chest requires aortic root surgery, the operator may face a major clinical challenge in selecting the optimal surgical access and approach. We present a case of a patient with Marfan syndrome and severe pectus excavatum who underwent aortic valve replacement, aortic root and ascending aorta reconstruction, coronary artery reimplantation (Bentall de Bono procedure ) via upper partial L-sternotomy and anterior left thoracotomy.