Diffuse pulmonary haemorrhage is an ominous condition that has a high paediatric mortality rate. Recombinant activated factor VIIa (rFVIIa) is a powerful haemostatic agent which has been used intravenously in life-threatening haemorrhage in variety of conditions in which conventional medical or surgical therapy are unsuccessful. We report off-label successful use of endotracheal rFVIIa for massive life-threatening respiratory haemorrhage following aspiration and cardiopulmonary resuscitation in a 3-month-old infant who was anticoagulated with enoxaparin following corrective cardiac surgery with other comorbidities. Off-label administration of endotracheal rFVIIa permitted rapid safe control of massive pulmonary haemorrhage and prevented further detrimental decline in respiratory function with satisfactory outcome.