The DS patient has clear anatomic differences in the head and neck region when compared with the general population. These anomalies include a flat occiput, oblique palpebral fissures, epicanthal folds, speckled irides, a protruding tongue, prominent malformed ears, and a flat nasal bridge. Congenital otologic anomalies and acquired conditions such as otitis media are also more frequently observed in the DS population. The DS patient is predisposed to obstructive sleep apnea, and the diagnosis of sleep apnea in DS patients is more likely to be delayed. A child with DS who has a narrowed nasopharynx, large tongue, and a subglottis which is smaller than normal must be given special consideration at the time of intubation. Such a patient requires an endotracheal tube two sizes smaller than the standard size appropriate for the patient's age. The child should also be suspected of having and be evaluated for obstructive sleep apnea, to ensure that appropriate precautions are taken in the perioperative period. Finally, any DS patient undergoing preoperative evaluation for a general anesthetic should have a careful assessment of the cervical spine to avoid dislocation or spinal cord injury. Hearing loss may be suspected in any congenital syndrome. In DS, there is a clearly increased incidence of congenital temporal bone anomalies, external auditory canal stenosis, and otitis media. All DS patients should undergo hearing assessment in the neonatal period, with follow-up as appropriate. Aggressive treatment of conductive hearing loss and early amplification may be necessary to maximize speech and language development.