Hairy polyps are rare congenital malformations that can arise in the nasopharynx. It is rarer for them to originate from the eustachian tube. They present with intermittent airway obstruction and respiratory distress. A thorough ear, nose, and throat examination can be required to identify these lesions. We present a case of eustachian tube hairy polyp causing neonatal respiratory distress in a 1-month baby successfully removed by a combined transoral and nasendoscopic resection.