Bronchopulmonary sequestration is a rare congenital pulmonary malformation. It is usually manifested as hemoptysis or recurrent pneumonia. On chest radiography, sequestration typically appears as a solid mass, a cystic lesion, or an air-fluid level, depending on the communication to the airway or the infection condition. However, pulmonary sequestration with rib notching on the chest radiograph has never been reported in the literature. Herein, we report a case of pulmonary sequestration with unilateral rib notching.