Situs inversus totalis (SIT) is a rare condition characterized by the mirror image location of all of the thoracic and abdominal organs. There are only a handful of reports documenting the presence and repair of an abdominal aortic aneurysm in the setting of SIT. Here, we present a rare case of a juxtarenal abdominal aortic aneurysm repaired through a retroperitoneal approach in a patient with SIT. We demonstrate that the retroperitoneal approach is a safe and effective method to manage complex aortic aneurysm disease in a patient with SIT.