A 14-year-old girl who presented with an endodermal cyst manifesting as severe neck and shoulder pain along with vesicorectal disturbances. Cervical magnetic resonance imaging showed a slightly enhanced intradural cyst at the C6-7 level in the ventral side of the spinal canal, with significant dorsal shortening and thinning of the spinal cord. Anterior corpectomy was chosen because of the dorsal effacement of the spinal cord. The cyst wall was subtotally removed to avoid damage to the normal spinal cord. After cyst removal, the iliac bone and an anterior cervical plate were used for anterior fusion. Postoperatively, her pain subsided without neurological deficits. The histological diagnosis was endodermal cyst. The cyst did not recur during a follow-up period of 18 months. Endodermal cysts are rare congenital lesions of the spine lined by endodermal epithelium. The natural history of this lesion is unclear, and the surgical strategy for the approach route and the extent of removal of the cyst wall remain controversial. We suggest that the anterior approach may allow a safer and more effective surgical route for the treatment of ventrally located endodermal cyst compared to the posterior approach.