Chylothorax is a rare but dangerous cause of respiratory failure in the pediatric population. It most commonly presents after cardiac surgery or, alternatively, due to retrograde pressure on the thoracic duct from narrowing or obstruction in the innominate vein due to thrombus or neoplasm. We observed an unusual presentation of chylothorax in an otherwise healthy 3-month-old infant with congenital superior vena cava stenosis leading to acute respiratory collapse. After initially not responding to medical therapy, the patient was successfully treated with an intravascular stent.