Vallecular cyst is a rare cause of stridor and respiratory distress in infancy and has been associated with sudden airway obstruction resulting in death. In a retrospective review of eight cases over a 20-year period all infants developed symptoms during the first week of life. All had stridor and feeding difficulties and two required endotracheal intubation. Other common findings included signs of increased work of breathing and episodes of cyanosis. Failure to thrive was present in five patients diagnosed later than the first week of life. An abnormal or a hoarse cry was present in only two patients. Diagnosis in all cases was made by endoscopy. Where a VC is clinically suspected, it is important to stress the need to visualize the base of the tongue during any diagnostic endoscopic procedure. At endoscopy, a smooth localized mass arising from and distorting the lingual surface of the epiglottis was identified. Histologically, the cysts contained respiratory epithelium with mucous glands with an external lining of squamous epithelium. Treatment by cyst marsupialization is safe and definitive, in particular when performed by CO2 laser.