RESUMEN
Ankyloglossia superior is an exceedingly rare congenital condition that consists of a connection between the tongue and hard palate. This abnormality is considered part of the ankyloglossia superior syndrome when found with other malformations such as limb deformities, gastrointestinal malformation, and cleft palate. Ankyloglossia superior can also be associated with other known syndromes. We have presented the case of a female infant born with multiple malformations, including partial agenesis of the feet and hands, micrognathia, a lack of expression of the facial muscles, convergent strabismus, mouth opening limitation, and tongue-palate adhesion. The patient's presenting diagnosis was ankyloglossia superior associated with Moebius syndrome. Computed tomography revealed the extent of the ankyloglossia superior and the loss of integrity of the palatal shelf. Surgical release of the ankyloglossia superior was performed with the patient under general anesthesia. Multiple management challenges were encountered postoperatively. To the best of our knowledge, ankyloglossia superior presenting in conjunction with Moebius syndrome had not been formally described in a case report.