Background: Cervical myelopathy is commonly seen in the older population caused by a degenerative process, but in a rare case, it can be seen in the pediatric population due to uncommon causes, such as an intraspinal cyst. This case report aims to discuss a rare case of cervical myelopathy due to an endodermal cyst in a two-year-old child. Case Presentation: A 2-year-old child with limited neck movement and abnormal body posture from 6 months before admission to the hospital. Spine trauma and other histories of disease were denied. There was no deformity, swelling, hematoma, and wound on the vertebra region from physical examinations. The motoric function was found weaker on the upper and lower right extremities, and pathological reflex was found on both sides. MRI examination showed an intradural extra-medullar space-occupying lesion on the C2-C5 level of the spine. At first, the patient was diagnosed with cervical myelopathy due to an intradural extramedullary tumor at level C2-C5. Excision of the cyst, posterior decompression, and laminectomy was done in this patient. The postoperative histopathological result showed an intraspinal cyst with an endodermal type cyst. Conclusion: This case study was first diagnosed due to an intradural extramedullary tumor, which appears to be a benign intraspinal cyst from the histopathological result. The surgical treatment results in a favorable outcome, but a routine followup should monitor the improvement or long-term neurological decline.