Goldenhar syndrome (also known as oculo-auriculo-vertebral spectrum, facio-auriculo-vertebral syndrome, and Goldenhar-Gorlin syndrome) is caused by fetal growth disturbances of the first two brachial clefts. Diagnostic criteria include eye, ear, mandibular, and/or vertebral anomalies. These patients may also have cardiac and renal malformations with varying degrees of severity. Airway management for Goldenhar patients may include difficult ventilation and intubation, which may become increasingly difficult with age. Vertebral anomalies including fused cervical vertebrae and/or cervical instability further complicate airway management. Pulmonary complications occur due to congenital malformations and scoliosis, which can lead to thoracic insufficiency syndrome. This chapter discusses genetics, presentation, and management of Goldenhar syndrome.