BACKGROUND: Spinal neurenteric cysts, also known as endodermal or enterogenous cysts, are rare epithelium-lined structures of presumed endodermal origin. Congenital vertebral anomalies are frequently seen in patients with neurenteric cysts, most typically anterior spina bifida, butterfly vertebrae, and hemivertebrae. However, few series of intraspinal neurenteric cysts accompanied by Klippel-Feil syndrome have been reported previously. CASE DESCRIPTION: Our purpose is to present the clinical, radiological, and histological results of a 29-year-old patient with a spinal neurenteric cyst associated with Klippel-Feil syndrome and to review previous reported cases of neurenteric cysts associated with Klippel-Feil syndrome. In our patient, cervical radiography demonstrated C5-T1 vertebral fusion and magnetic resonance imaging revealed a large intradural cystic mass. The cystic lesion was removed successfully, and it was histopathologically diagnosed as a neurenteric cyst. CONCLUSION: Neurenteric cysts should always be considered in the differential diagnosis of an intraspinal cystic mass seen in the setting of vertebral anomalies.