Bronchial atresia (BA) is a rare congenital pulmonary airway malformation. It is characterized by the focal stenosis of a proximal segmental bronchus associated with peripheral mucus impaction and hyperinflation of the obstructed lung segment. Most cases are identified during neonatal period or childhood. When diagnosed in adults, BA may present with recurrent infections, pneumothorax and destruction of affected parenchyma. Thoracoscopic approach to BA has proved challenging in adult patients because of repeated infections and subsequently, its inflammatory status. Herein we present a case of a 26-year-old female with left side recurrent pneumonia and pneumothorax past history. A chest computed tomography revealed a complex congenital bronchial atresia involving the left upper lobe and basal segments, associated to vascular anomalies. She underwent a successful uniportal VATS left upper lobectomy and resection of basal segments. Uniportal VATS approach is an effective and safe treatment for the management of complex congenital lung malformation.