Spinal neurenteric cyst results from the persistence of an abnormal communication between endodermal and neuroectodermal layer. It is a rare malformation that lead to spinal cord compression. The patient is a 22-month-old boy with sudden, progressive left side motor weakness. Preoperative magnetic resonance image revealed intradural extramedullary cyst in C4-C5 level that compressed the spinal cord on the left side. After the resection of the cyst, motor weakness of the left side was partially improved, and his motor power was completely recovered after rehabilitation. Histologic examination revealed a ciliated columnar epithelial neurenteric cyst. The pre- and postoperative clinical features of the case of neurenteric cyst is described with the review of literature.