As an uncommon subtype of meningioma, chordoid meningioma (CM) of the spinal canal is extremely rare. There have been only two reported cases of intraspinal CM in the literature, and this lesion has not been previously reported in the pediatric age group. To the best of our knowledge, the present study reports the first case of spinal chordoid meningioma in a pediatric patient. A 12-year-old female presented with a 3-month history of progressive numbness and weakness in the right-side limbs, and intermittent pain in the neck and right shoulder. Spinal magnetic resonance imaging (MRI) revealed an intraspinal lesion at the C2-3 level with irregularly heterogeneous enhancement. The patient underwent a C2-3 laminotomy. Due to adhesion to the dura and proximity to the vertebral artery, the tumor was partially removed intraoperatively. The post-operative course was uneventful and the symptoms were apparently relieved. The patient experienced recurrence 5 years subsequent to surgery. MRI revealed an extradural regrown tumor at the C2-5 level. Partial removal combined with radiotherapy was performed. However, the patient experienced progression of tetraplegia and succumbed to severe pneumonia and respiratory failure 5 months subsequent to the second surgery. In the present study, the clinicoradiological findings and treatment outcome of this rare entity are discussed, in addition to a review of the relevant literature. Spinal CMs should be included in the differential diagnosis of intraspinal tumors of the pediatric spine. Multidisciplinary treatment, consisting of total surgical removal and adjuvant radiotherapy, should be considered due to the aggressive nature of this abnormality and the risk of long-term recurrence.