Abstract
Herein, we describe a 21-month-old girl with PHACE syndrome (posterior fossa hemangiomas, arterial lesions, cardiac anomalies/coarctation of the aorta, and eye anomalies) who presented with a tortuous extensive aortic arch aneurysm. As the maximum short diameter of the distal aortic arch aneurysm expanded rapidly from 21 mm to 25 mm in only 5 months, we performed extensive aortic arch reconstruction with interposition graft replacement through a left thoracotomy under partial cardiopulmonary bypass.