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We present a case of a 16-year-old adolescent female with blood group O+ who was diagnosed with cystic fibrosis (CF). The patient had to be hospitalized due to septic shock and respiratory failure, and extracorporeal membrane oxygenation and mechanical ventilation were applied. Faced with high urgency, she was promptly enlisted for a lung transplant, ultimately receiving a blood group A1 deceased donor lung through rescue allocation. Bilateral incompatible lung transplantation, with parental consent, was successfully performed. The postoperative course was favorable, marked by the administration of rabbit anti-thymocyte globulin, plasmapheresis, and immunosuppression (mycophenolate, steroids, and tacrolimus) as per the prescribed protocol. Notably, the patient experienced a smooth recovery without infectious complications or humoral rejection. This case highlights the viability of lung transplantation in cases of ABO incompatibility, particularly for patients in urgent need on the transplant waiting list., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Varón-Vega et al.)