Cervical split cord malformations are extremely rare with less than 75 cases reported in the literature worldwide. The widely different terminologies used to describe the same pathoanatomy make the documentation of all reported cases a difficult task. We conducted a systematic review of 71 cases documented over 38 reports from 1889 to 2016. The controversy regarding prophylactic surgery in asymptomatic individuals remains. We advocate prophylactic surgery in all asymptomatic individuals to preclude severe neurological deficit following trivial trauma in future. The results of surgery in asymptomatic individuals are excellent while those in symptomatic individuals are good as well.
Keywords: Cervical spine; diagnosis; split cord; treatment.