We report a female infant with presentation of epignathus teratoma involving duplication of both the mandible and tongue. Epignathus with duplication of the mandible has rarely been reported in the literature thus far. The location and extent of the tumor, as well as the involvement of adjacent structures, resulted in trismus and upper airway obstruction at birth. Thus, staged operations including debulking and correction of anatomical anomaly were performed on this patient after life-saving tracheostomy. As a result, we not only prevented morbidity associated with the anomaly but also refined the patient's appearance and improved her quality of life.
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