Fourth branchial pouch anomalies are extremely rare and only a few such cases showing sinuses and cystic masses have been reported in the literature. We describe a patient who presented on the third day of life with cystic neck swelling of fourth branchial pouch origin giving rise to respiratory obstruction and stridor. Despite repeated aspiration of the cystic mass to relieve respiratory obstruction, rapid recurrence of the mass continued to cause stridor and ultimately required surgical excision. The clinical, radiological, and histopathological findings of this unusual condition are discussed with a review of the literature.