The incidence of neurenteric cyst (NC) is rare amongst spine tumors. It is most often asymptomatic but may present with sensory and motor symptoms. When associated with thoracic vertebra fusion it is not reported before, this complicates the placement of pedicle screw during posterior instrumentation. Herein, we report a case of thoracic spinal neurenteric cyst in a 40-year-old man that presents with chronic back pain, left lower limb weakness and numbness. Elective excision of NC over T6-T7 with laminectomy and multilevel posterior instrumentation was successfully performed with significant improvement of the symptoms. Neurenteric cyst is a rare spinal cord lesion which may cause permanent neurological sequalae. Complete surgical excision with spine fixation in this case provides good long-term outcome.