Epignathus or Tessier fissure 0-14 is a rare teratoma composed of the three embryonic germ layers. Clinically, it appears as a mass covered with skin and mucosa, originating from pluripotential stem cells. According to the latest classification by the World Health Organization teratoma represents the main benign Germ cell tumors of the sinus region and presents similar histological aspects with its counterparts in the gonads and other extragonadal sites. The purpose of this study is to present a case of epignathus originating from the base of the skull in a Caucasian female patient with a cleft palate after the incisive foramen, not affecting the nasal floor. During physical examination, via intraoral route, in the region below the base of the skull, an extensive solid mass, covered by mucosa and with the presence of hair. Computed tomography confirmed the lesion, in the region corresponding to the base of the patient’s skull. The first surgical approach of resection occurred at the 40th day of the patient’s life, under general anesthesia and subsequent primary closure of the palatal defect. The second surgery was performed 10 months later, when tissue expanders were used on the palate to increase the mucosal surface. At the age of 5, the patient underwent surgery with a tongue flap, from the lingual dorsum, with an anterior pedicle. The patient was kept under sedation and relaxation for a period of 10 days for flap autonomy. After this period, the section of the flap and the reconstruction of the tongue dorsum were performed. The patient had a good evolution and was maintained under outpatient treatment. Pediatricians and pediatric dentists should be aware of the importance of prenatal exams and soon after birth, to identify possible deformities in neonatal patients, which favors the diagnosis. The 17-year postoperative follow-up of complete surgical removal of the teratoma and reconstruction of the cleft palate, combining the use of a tissue expander on the palate and a flap on the dorsum of the tongue, proved to be a viable resource for improving the patient’s quality of life.
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