A 16-year-old boy with Chiari 1 malformation presented for an elective suboccipital craniectomy and C1 laminectomy. His intraoperative course was uneventful. At the conclusion of the procedure, he met extubation criteria and followed commands. After extubation, he developed progressive upper airway obstruction and became obtunded. He was reintubated via videolaryngoscopy, which showed edema not only to the tongue, but also to the posterior pharynx and blisters over the vocal folds and epiglottis. The patient was transferred to the pediatric intensive care unit intubated and sedated. This report describes the clinical course of his massive macroglossia and discusses short- and long-term management.