Two patients with oculo-auriculo-vertebral syndrome and multiple airway anomalies are presented. Both patients had esophageal atresia with a distal tracheoesophageal fistula and tracheomalacia due to innominate artery compression. Pulmonary hypoplasia, obstructive sleep apnea, and a laryngeal anomaly were also noted. The literature of airway anomalies and obstructive sleep apnea described in association with the oculo-auriculo-vertebral spectrum is reviewed.