The case of a six-day-old neonate admitted in an emergency situation because of dyspnea and increasing cyanosis is reported. Despite abnormal opacification on the chest X-ray and left ventricular overload on the electrocardiogram and echocardiogram, features compatible with the disease, the diagnosis of massive pulmonary arteriovenous fistula affecting the whole left superior lobe, was made possible only after necroscopic examination.