Preterm infants are at risk for developing bronchopulmonary dysplasia (BPD) and chronic lung disease (CLD). Pulmonary malformations are typically congenital or occur in the elderly adult populations. This case is of a 6-month-old infant who was born at 23 weeks gestation. At 3 months of life, the infant developed a clinically significant right sided lobar emphysema. This infant had no know congenital pulmonary malformations. Right upper lobectomy was performed. There are multiple reported cases of neonates requiring a lobectomy for congenital pulmonary malformations. This case demonstrates improved clinical outcome for an extremely preterm infant after surgical resection of an acquired lobar emphysema (ALE).