The proper management of upper airway obstruction requires prompt recognition, correct diagnosis, and appropriate treatment. Of all the causes of upper airway compromise, intratracheal thyroid is one of the least common, especially in the newborn. The majority of cases reported involve adults or adolescents, mostly in European countries. The English literature has fewer cases, none involving a newborn. The authors report a case they believe to be the first example in the English language of obstructing intratracheal thyroid in a newborn. The authors' experience and literature review of this rare anomaly are presented in an effort to acquaint others with this often unthought of cause of airway compromise.