Acute aortic dissection is a life-threatening condition. We report the case of a neonatal one-stage correction of coarctation and hypoplastic aortic arch repair plus ventricular septal defect closure. While dissecting the head vessels after cannulation of the ascending aorta and commencing cardiopulmonary bypass, type A aortic dissection evolved. This required immediate ascending aorta and aortic arch reconstruction with coarctation of the aorta resection under hypothermic circulatory arrest. The surgical management of this rather unique situation is discussed here.