Mucocoele of the Blandin-Nuhn glands and teratomas of the tongue are rare in newborn babies. We present what is to our knowledge the first documented case of both at the same time, and describe management and follow up over 12 months after the birth. Prenatal diagnosis defined the treatment plan during delivery to establish an airway for the baby promptly. A normal delivery is possible if the cyst is anterior, and can be punctured during delivery. Delayed excision reduces the possibility of oedema and obstruction of the airway, ensures complete resection, avoids recurrence, and confirms the diagnosis. Mucocoeles are classified as extravasation or retention types; our observation supports the hypothesis of a "malformative type". A long follow-up is essential to monitor macroglossia, open bite, and the development of the mandible.
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