The clinical approach to managing high-grade arteriovenous malformations (AVMs) has been challenging due to its various presentations, surgical risk of complications, and impact on patients’ quality of life. We report a case of a 57-year-old female who experienced recurrent seizures and progressive cognitive decline secondary to a grade 5 cerebellar AVM. We reviewed the patient’s presentation and clinical course. We also searched the literature for studies, reviews, and case reports involving the management of high-grade AVMs. We outline our recommendations on how to approach these cases after a review of the currently available treatment options.