We report a first case with the use of extracorporeal carbon dioxide removal system as a bridge to re-do lung transplant in complete situs inversus patient. A 29-year-old female with Kartagener syndrome and complete situs inversus underwent a double lung transplant for end stage lung disease. Within one year after transplant the patient had primarily hypercapnic respiratory failure with radiographic signs of chronic lung allograft dysfunction. To optimize her nutritional status and muscle strength before re-do lung transplantation, we decided to bridge her with an extracorporeal carbon dioxide removal system due to anatomical difficulty. She was listed and underwent an uneventful re-do double lung transplant with cardiopulmonary support.