Objectives: This study aims to evaluate the clinical presentation, diagnosis, and management of infants with a congenital vallecular cyst (VC).Patients and Methods: Four infants (2 males, 2 females; mean age: 45±17 days; range, 28 to 63 days) who were operated or followed for a congenital VC were retrospectively analyzed between January 2016 and March 2020. Data including patient demographics, major symptoms (cyanosis, inspiratory stridor, suprasternal retraction, difficulty feeding), diagnostic methods, type of coexisting anomalies, surgical procedures, and length of follow-up and recurrence were recorded.Results: All patients presented with varying degrees of respiratory symptoms including inspiratory stridor and suprasternal retraction. The diagnosis was made based on awake flexible fiberoptic laryngoscopy (FFL) in all four infants. Two patients were treated with endoscopic cyst excision. One case underwent endoscopic cyst excision and supraglottoplasty, while the other was followed without surgery. All surgeries were successful, as none of the patients experienced recurrence.Conclusion: Although congenital VCs are extremely rare, they should be considered among the differential diagnosis of congenital stridor. A detailed examination with FFL should be performed for diagnosis. Based on our findings, transoral endoscopic excision of VCs is a safe, effective, and feasible procedure as a surgical treatment to prevent recurrence.