Purpose: Neonates and infants have an unusual spectrum of end stage pulmonary diseases distinguishing them from other patients undergoing bilateral lung transplant (BLT). This study was undertaken to review a single institutional experience with BLT in infants 1 year of age or less.Procedures: A retrospective review was performed of pediatric patients who underwent BLT between January 1990 and September 2002.Results: A total of 207 children under the age 18 years underwent primary BLT, 34 (16.4%) of which were infants. Major disease entities prompting BLT included pulmonary hypertension of varying etiologies (n=13), pulmonary alveolar proteinosis (n=11), and infantile interstitial pneumonitis (n=5). Prior to transplant, 10 patients were on ECMO and 27 were on mechanical ventilatory support. Median wait list time was 22 days (2–15 days), median age at transplant was 127 days (36 days–1 year) and median weight was 5.2 kg (3.2–11.8 kg). Median length of ventilatory support post-transplant was 16.5 days (2–89 days) and median hospital stay was 20 days (4–79 days). Hospital mortality of 24% (8/34). Acute rejection was rare with an average of only 0.2 episodes per patient despite routine surveillance biopsies. One and 5-year survivals were 70% and 53% respectively with a mean follow-up time of 4 years. Freedom from bronchiolitis obliterans (BO) at 1 and 5 years was 92% and 78% compared to 80% and 43% in the other 173 lung transplants performed during this interval (p=0.02). Two retransplants were performed both within 6 months of primary transplant, one of whom is a long-term survivor.Conclusions: Acceptable mortality and long term survival can be achieved in infants undergoing BLT. As incidence of BO is significantly decreased in this patient population, avoidance of factors contributing to early mortality should lead to long-term survival that supersedes that seen in older patients undergoing BLT.