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A 4.1 kg male neonate with a diagnosis of double-inlet left ventricle with dextro-transposition of the great arteries was intubated shortly after birth due to respiratory insufficiency. The initial management consisted of a successful Stage I hybrid procedure. Persistent respiratory insufficiency led to cross-sectional imaging and bronchoscopy that demonstrated severe airway compression from a dilated main pulmonary artery. A Norwood procedure with Blalock–Thomas–Taussig shunt was performed at 1 month of age to relieve the airway obstruction. The patient was discharged home on room air at 2 months of age. This case highlights a unique single-ventricle anatomic variant with airway compression, which was successfully managed with deferred Norwood palliation.