There are various causes of possible upper airway obstruction in infants. Particularly, large cysts on the base of tongue may cause severe airway obstruction by a mass effect on the hypopharynx and by displacing the epiglottis. Of these basal lingual cysts, thyroglossal duct cyst is rare but occasionally its remnants can be found at the base of the tongue. Although they are pathologically benign, basal lingual cysts may result in death by asphyxia. There are a few reported cases of asphyxia caused by basal lingual cyst. All died suddenly in the bed or cot while sleeping without any significant symptoms and signs. The authors experienced a unique case of death due to airway obstruction by basal lingual cyst (thyroglossal duct cyst), confirmed by postmortem examination, probably aggravated after central catheterization in 2-month-old infant who had open heart surgery for atrial and ventricular septal defect and report with a literature review.