We present a case of pulmonary atresia with intact ventricular septum and suspected right ventricle-dependent coronary circulation in a full-term neonate, highlighting the diagnostic contribution of computed tomography (CT) in evaluating a rare constellation of cardiac and airway anomalies. CT imaging demonstrated markedly dilated coronary arteries, right ventricle hypertrophy and hypoplasia, a tortuous patent ductus arteriosus causing airway compression, and complete pulmonary atresia. This complex anatomy significantly influenced the surgical decision-making process. The case illustrates CT's role in delineating both intracardiac and extracardiac anatomy relevant to perioperative planning.