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A carotid cavernous fistula (CCF) is a disruption in the carotid arteries within the cavernous sinus. The pooling of blood in the sinus causes a myriad of neurological deficits. When correctly diagnosed, this condition can be easily managed through surgical intervention. However, its rareness and similarity in presentation to other neurological conditions allow for misdiagnosis and subsequent progression to permanent disability. Therefore, it is imperative to understand the unique clinical features and signs of CCFs. We report on the clinical workup, diagnosis, and treatment of the first documented case of a patient who developed a carotid cavernous fistula in conjunction with spine surgery., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sharkey et al.)