eng Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2021-04-29 9 8 407 410 10.12691/ajmcr-9-8-6 AJMCR2021986 article Sharif Al Qadhi 1 Simin Laiq 2 Neeraj Salhotra neersal@hotmail.com 2 Jospaul Lukas 3 Zahra Al Hajri 3 Bashar Al Tunbi 3 Fahad Al Kheder 3 Mohammad Hashim 3 Muna Al Jabri 3 Afaf Rashid Al Hatmi 3 Department of Neurosurgery, Khoula Hospital, Muscat, Oman Department of Neuropathology, Khoula Hospital, Muscat, Oman Department of Neuroradiology Neuropathology, Khoula Hospital, Muscat, Oman Neurenteric cysts, also known as endodermal cysts and enterogenous cysts, are rare benign cysts. Neurenteric cysts may be associated with congenital malformations, such as spinal dysraphism, or may occur as solitary lesions. Most are located in the spine, in the intradural, extramedullary and intraspinal space. Rare examples may occur intracranially, and rarer examples may occur at the craniovertebral junction. Clinically, solitary neurenteric cysts present with mass effect symptoms such as compression of spinal cord and adjacent nerve roots. We present a case of a 28-year-old male patient with a histologically confirmed neurenteric cyst located at the cervicomedullary or craniovertebral junction with presenting symptoms of neck pain and severe debilitating weakness of all four limbs. The patient underwent successful surgical resection, with gradual improvement in his symptoms. We also present a review of literature, including the clinical features, histological characteristics, and management options for patients with neurenteric cyst in the craniovertebral junction. http://pubs.sciepub.com/ajmcr/9/8/6/ajmcr-9-8-6.pdf Neurenteric cyst enterogenous cyst cervicomedullary junction craniovertebral junction far-lateral approach