A neonate with unbalanced right-dominant atrioventricular canal defect with intact atrial septum and abnormal pulmonary venous return developed bradycardic arrest and a subsequent diagnosis of tracheal rings and left lung aplasia. This report details the fatal nature of the triad of hypoplastic left heart, pulmonary venous obstruction, and airway anomalies, along with the role of advanced imaging in prognostication.