Macroglossia, an abnormal swelling of the tongue, is a rare post-operative complication often associated with serious airway obstruction and prolonged intubations. Currently, there is a paucity of information on the true incidence, aetiology, and complications associated with macroglossia. A thorough review of the literature was carried out so as to summarise the characteristics of reported cases of macroglossia and to present potential treatments and preventive strategies. A literature search was conducted in PubMed to identify human case reports of macroglossia after neurosurgical procedures including spine, published in English from 1974 to December 2015. A total of 26 reports with 36 cases of macroglossia were identified. Macroglossia was most commonly reported after sub-occipital and/or posterior fossa craniotomies and spine surgeries in prone or park-bench positions. It is more common after procedures lasting >8 h. The aetiology of macroglossia is multi-factorial and possible mechanisms included local mechanical tongue compression interfering with venous and/or lymphatic drainage, regional venous thrombosis and/or local trauma. Complications included airway obstruction, re-intubation, difficult re-intubation, prolonged intubation and Intensive Care Unit stay and tongue necrosis. Prevention, awareness of the possibility, and early recognition are the best forms of treatment.