A 36-year-old female patient with a long-standing asymptomatic lower thoracic scoliosis presented with sensory symptoms involving all limbs. MRI scan demonstrated a rounded ventral intradural mass causing major deformity of the cervical cord at C6 and C7 levels. Unlike most previously reported neurenteric cysts, the MRI signal characteristics of this mass were such that it could not be determined if it is cystic or solid, being iso-intense on T1- and hyperintense T2-weighted images. Resection was performed through a median corporectomy of C6 and C7, the lesion being found to be a neurenteric cyst with an attachment to the anterior median fissure of the cord. Strut graft and cervical locking plate fixation from C5 to C6 was facilitated by extending the cervical incision into the sternal notch, with detachment of left-sided strap muscle insertion. The patient made an excellent recovery with complete resolution of neurological symptoms and solid fusion. The postoperative course was complicated by an anterior cervical CSF collection which resolved spontaneously within 2 months. The literature regarding this rare condition and its management is reviewed. Although the majority of intraspinal neurenteric cysts are situated ventral to the cord, most reports of excision have been from a dorsal approach. Drainage and subtotal excision of neurenteric cysts have been previously advocated; however, the recurrence rate is such that complete excision is advocated. This is facilitated by a ventral approach. A simplified method of utilising the sternal notch exposure is reported. The literature regarding the anatomical peculiarities pertinent to the sternal notch approach, and the reported literature regarding spinal neurenteric cysts is reviewed.