Intestinal transplantation (IT) is a definitive therapy for children with intestinal failure, yet postoperative care and ventilatory management are challenging. Acute respiratory distress syndrome (ARDS) is a severe complication of IT with high mortality in children. We report a case of a 10-year-old girl who developed ARDS after IT. She was successfully managed with lung protective strategies and early inhaled nitric oxide therapy. The use of PEEP on patients after IT should be cautiously undertaken due to high intra-abdominal pressure.