AIM: Caused by a disturbance in the neurulation process, neural tube defects (NTD) are easily diagnosed and treated in the early years of life. Although early repair of NTD is advocated, there is lacking information on its natural course. There are only 11 cases, including this one, reported in literature of a cervical meningocele diagnosed and treated in an adult. CASE PRESENTATION: A 64-year-old male patient was aware of a skin lesion on his neck but had not consulted a physician until 3 years ago when he started experiencing neuropathic pain in his arms. Cervical MRI revealed a posterior arch fusion defect, a syrinx cavity between the C2-C6 levels with a tethering meningocele at the C6 level. He was operated on with the excision of the skin tag and connecting stalk. During prone positioning and neck flexion, intraoperative neuromonitorazion indicated a loss in MEP values; thus, flexion was reduced. Intradural adhesiolysis of the connecting bands around the spinal cord was performed, and dura was sutured. The patient recovered uneventfully, but he still had neuropathic pain in his left arm aggravated by straining. Histological analysis revealed meningothelial cells and psammoma bodies. Postoperative MRI revealed the untethering of the spinal cord with relative reduction in the syrinx cavity. CONCLUSION: NTD can be easily detected and treated early in life to prevent neurological deterioration, to decrease the rate of infection, and to improve cosmetic appearance. However, because they are mostly treated during childhood, their natural course in adulthood remains unknown. There are only a handful of case reports of cervical meningoceles in adults. Pathological analysis suggestive of a meningioma may also shed light on the theory of “cutaneous meningioma.” In addition, the loss of MEP values in neck flexion should caution surgeons in avoiding hyperflexion, especially in cases of spinal cord tethering.