Intracranial neurenteric cyst at the anterior craniocervical junction is very rare, and its treatment and prognosis have not been established. We report a case of neurenteric cyst at the anterior craniocervical junction and review the relevant literature. A 16-year-old girl presented with a 2-month history of slowly progressive headache. MRI revealed a well-defined intradural extramedullary cyst in the anterior medulla and brain stem with C1 cord compression. We performed gross total resection of the cyst using a far-lateral transcondylar approach. Surgical resection is the treatment of choice for neurenteric cysts at anterior craniocervical junction, the far-lateral transcondylar approach might be the optimal surgical approach.