Background:As chordomas are slow growing and locally invasive with high recurrence rates,initial recommendations include complete surgical resection with or without radiation therapy.A large proportion of recurrences occur years after initial resection necessitating lengthy follow-up.The novel biomarker brachyury and the repurposing of pharmaceutical products have the potential to substantially impact long-term recurrence rates.Case presentation:A 43-year-old woman presented with an isolated,cervical extraosseous intradural extramedullary chordoma attached to a nerve root underwent a C3-5 laminectomy,C3-5 lateral mass screw instrumentation,and mass resection.All symptoms resolved by the 12-month postoperative follow-up visit.Conclusions:This is the first report of an isolated,cervical extraosseous intradural extramedullary chordoma attached to a nerve root,and this case adds to the previous six Type Ⅳ chordomas in the literature.Unfortunately,the very rare form of extraosseous intradural chordoma is poorly understood:the lack of detailed knowledge in how they are differentiated from other forms of chordoma confounds the development of optimal treatment strategies and follow-up guidelines.