Spondyloepiphyseal dysplasia congenita (SEDC) is a type of short-limbed dwarfism characterized by platyspondylia, delayed metaphyseal ossification, and irregularly shaped bones. Anesthetic issues in patients with SEDC have reportedly included airway stenosis caused by laryngotracheal hypoplasia, ventilation difficulty due to facial hypoplasia, and intubation difficulty attributed to microgenia. Furthermore, atlantoaxial instability can lead to cervical dislocation due to excessive or violent manipulation of the head and neck. We present the case of a 5-year-old girl with SEDC scheduled for palatoplasty revision. Airway difficulties were anticipated because of microgenia and the cervical collar she wore for atlantoaxial instability. However, mask ventilation and video laryngoscopy proved relatively easy. The patient was placed in Trendelenburg position (approximately 10°) without head tilt for surgical access. A combination of formulas based on the patient’s age and height was used to determine tracheal tube size. However, the 4.5-mm oral Ring, Adair, Elwyn (RAE) tube selected resulted in 1-lung intubation when the tube bend was fixed at the lip, requiring further depth adjustment. Successful anesthetic management of this patient with SEDC incorporated several factors, including an individualized airway management plan, use of a video laryngoscope, careful posturing to avoid excessive cervical strain, and appropriate tube sizing and positioning.