Background: Eagle syndrome (ES) is a rare symptomatic condition generally caused by an elongated styloid process (SP) or calcification of the stylohyoid complex. On the diagnosis is made, its treatment remains subjective since the indications for surgical intervention are still not standardized. Although styloidectomy is the surgical treatment of choice, no consensus exists regarding the transcervical or/and transoral route. Here, we report our experience in a patient with bilateral internal carotid artery (ICA) dissection caused by ES, who underwent innovative surgical technique. Case Description: A 53-year-old man, with the right-sided middle cerebral artery acute stroke, underwent computed tomography angiography 3 days after a successful endovascular treatment. The study showed a bilateral ICA dissection with bilateral hypertrophic SPs and a close relationship of ICAs with both SPs anteriorly and C1 transverse process posteriorly. Considering the occurrence of ICA compression by a styloid/C1 transverse process juxtaposition, the patient underwent the left partial C1 transversectomy by an extraoral approach. A temporary paresis of the ipsilateral lower lip lasted 1 month, with a partial remission after 3 months. The patient reported significant improvement of symptoms with a good esthetics and functional outcome. Conclusion: A styloid/C1 transverse process juxtaposition should be considered as an alternative pathogenetic mechanism in vascular ES. When a posterior ICA compression by C1 transverse process is present, a bone reshaping of C1 rather than a conventional styloidectomy can be considered an efficacious treatment which allows a good preservation of the styloid muscles and ligaments. ©2019 Published by Scientific Scholar on behalf of Surgical Neurology International.