An 8-month-old female baby presenting with neck stiffness was found to have an intradural extramedullary cystic mass at C3 to T2 level on magnetic resonance images (MRI). The spinal cord posterior to the mass was compressed. Surgery and pathology revealed endodermal cyst. Repeated surgery was performed 14 months after the first operation because of local recurrence. The 27 months of clinical course after the second surgery was uneventful.