Neonatal upper airway obstruction is a life-threatening condition and requires rapid assessment and effective treatment. Malformations of the upper airway of a newborn can lead to acute respiratory distress. The aim of the present paper is to report the case of a newborn with respiratory distress due to a tongue base mass, which was removed surgically. The patient needed a tracheostomy tube for only three days and then could breathe spontaneously. However, he had swallowing problems, which decreased gradually over 9 months. Histopathologically, the mass was found to consist of mucous salivary glands, and was recorded as an ectopic salivary gland, which is extremely rare among the types of masses that may cause upper airway obstruction in a newborn.