Pulmonary hemorrhage is a rare but well-known complication in preterm infants. We present a case of massive pulmonary hemorrhage in a 9-day-old male infant, successfully treated with intravenous recombinant activated factor VII (rFVIIa) (NovoSeven; Novo Nordisk). The infant was diagnosed with sepsis-related disseminated intravascular coagulation and required ventilator support for respiratory distress syndrome and blood transfusions due to active bleeding from endotracheal tube. After administration of the second dose of rFVIIa (120 mu g/kg per dose, every 2 h), the active bleeding subsided dramatically and a significant improvement in the oxygenation index was seen 8 h after the third dose of rFVIIa treatment. There were also significant improvements in the prothrombin time, International Normalized Ratio, activated partial thromboplastin time and plasma fibrinogen levels after the third dose of rFVIIa treatment. The infant was discharged on day 82 of life and there was no finding of thrombosis during the hospitalization period. At month 18 of follow-up, there was no morbidity related to the pulmonary and central nervous systems. This case suggests that rFVIIa is effective as an alternative therapy in controlling massive pulmonary hemorrhage of preterm infants.