Pediatric lung transplantation is the most aggressive therapeutic option for children with end-stage pulmonary disease, but it remains a relatively rare operation. Major diagnoses necessitating transplant vary according to recipient age group. Cystic fibrosis is the most common indication in children ?6 years, while pulmonary hypertension and surfactant disorders account for the majority of cases in infants 1 year of age. Lungs are unique in that they remain directly exposed to the external environment with its infective and immunologic challenges and therefore high levels of immunosuppression are required. Acute rejection affects the majority of lung transplant recipients and typically is seen during the first 3 months post transplantation. It may be asymptomatic or present with nonspecific symptoms mimicking infection, including cough, fever, hypoxemia, tachypnea, or dyspnea. This chapter discusses the perioperative management of a patient post lung transplantation with symptomatology for diagnostic evaluation.