A 6-week-old female was referred to our unit because of increasing respiratory distress. The echocardiography (A and B, Online Videos 1 and 2) showed a large aorto-pulmonary window (APW, arrow in A) with a broad communication between the aorta (AO) and pulmonary artery (PA), anomalous origin of left pulmonary artery (LPA) from the right pulmonary artery with retrotracheal course (asterisk in B), and aberrant right subclavian artery. Computed tomography (C and D), bronchography (F), and flexible bronchoscopy (G) were used to confirm the diagnosis and further demonstrated tracheal compression. The origin of the right upper lobe bronchus (F, red arrow) and an aberrant right subclavian artery (E, asterisk) were also identified. Tracheal optical coherence tomography (H) was also performed and confirmed complete tracheal rings (translucent ring) surrounding both the anterior (arrow) and the posterior (arrow) portion of the trachea. The patient underwent surgical repair, and the intraoperative findings confirmed the diagnosis (I, J, K).