Transfusion-related acute lung injury (TRALI) is a frequently under-diagnosed, although potentially fatal, condition that represents a leading cause of transfusion-related morbidity and mortality even in pediatric patients. Its main clinical features are characterized by rapidly evolving respiratory distress, hypoxia, pulmonary edema, and bilateral infiltrates on chest radiograph during or within 6 h of transfusion. We present a case of severe TRALI associated with myocardial stunning that occurred in a 14-year-old girl, and review the existing literature of pediatric TRALI. Our report suggests a potential role for NIV in the management of TRALI as the best profile both in terms of safety and effectiveness for hematologic patients.