Sternal defects combined with craniofacial vascular defects are rare. We report on a 45-year-old woman with a sternal cleft associated with craniofacial and brain hemangiomata, an aneurysm of the aortic arch, anomalous origin of the coronary arteries, a left superior vena cava, micrognathia, supraumbilical midline raphe, and a cervical cyst. The surgical procedure consisted of the resection and replacement of the aortic arch and the innominate artery with reimplantation of the left carotid artery into the graft under circulatory arrest and deep hypothermia. The presence of sternal cleft is an indication for the search for other asymptomatic internal vascular anomalies.