Neurenteric cysts (NEC) are rare benign tumors of the central nervous system. Even more rare are the NEC at the ventral foramen magnum in children that are misdiagnosed. They cause distortion of the vertebrobasilar vascular system and cervicomedullary neural structures and are adherent to the latter. The inferior portion lies ventral to the upper most dentate ligament. This has implications in the surgical approach for complete excision.