Background: Eagle syndrome is a wide range of symptoms caused by elongated styloid process or ossified stylohoid or stylomandibular ligament compressing on surrounding structures. Internal jugular vein (IJV) compression from styloid process is a rare variation which leads to cerebral venous drainage and venous congestion or thrombosis. Case: A 25-year-old male presented with two tonic–clonic seizures. CT and MRI showed a dilated right cortical vein without haemorrhage. DSA confirmed a partially thrombosed cortical vein presumed secondary to a dural arteriovenous fistula. He was treated medically with levetiracetam and apixaban. Repeat MRI showed cortical vein recanalisation with persistent venous ectasia. Neuroradiology MDM identified severe right IJV stenosis likely secondary to an elongated styloid process, believed to be the culprit of impaired venous outflow and secondary cortical vein thrombosis. Right styloidectomy was subsequently performed through a transcervical approach. His cranial nerves were intact. He remained seizure-free and anticoagulation was ceased. Discussion: There are only few cases reporting venous variant of Eagle syndrome with cortical vein thrombosis and seizure. The condition is often misdiagnosed as a vascular malformation or idiopathic thrombosis. This is also the first case on the literature of unilateral Eagle syndrome with IJV stenosis requiring styloidectomy. Conclusion: IJV compression by the styloid process can mimic intracranial vascular malformations. Awareness of this entity enables timely medical management and may prevent future thrombotic or epileptic events. Reference: 1.Tadjer, J. and Béjot, Y., 2024. Vascular variant of Eagle syndrome: a review. Frontiers in Neurology, 15, p.1463275. 2.Zamboni, P., Scerrati, A., Menegatti, E., Galeotti, R., Lapparelli, M., Traina, L., Tessari, M., Ciorba, A., De Bonis, P. and Pelucchi, S., 2019. The eagle jugular syndrome. BMC neurology, 19(1), p.333.