Introduction. Tongue base cyst is an uncommon but potentially dangerous cause of stridor in neonates and infants. Case Presentation. We report a case of a 2-month-old Arabic male infant with a congenital tongue base cyst revealed by inspiratory stridor and recurrent respiratory distress. Diagnosis of cyst was suspected at endoscopy and confirmed by MRI imaging. The cyst was marsupialized with CO(2) laser. One year later, the child remains asymptomatic without recurrence of the mass. Conclusion. Tongue base cysts should be considered in differential diagnosis in new borns with stridor, respiratory difficulties, or swallowing problems. Definitive therapy requires large marsupialization under general anesthesia.