Background: Eagle syndrome is a condition that causes pharyngeal pain, facial pain, swallowing difficulties, and symptoms of arterial impingement due to the elongated styloid process. However, few reports were about eagle syndrome with venous compression up to now. This study aimed to identify the clinical profiles of the internal jugular vein stenosis (IJVS) related eagle syndrome comprehensively. Methods: A total of 27 patients, who were diagnosed as IJVS induced by styloid process compression were enrolled. The clinical manifestations and imaging features were analyzed. Results: Styloid process compression was presented in all of the 27 IJVS patients, in which, the top three symptoms included insomnia (81.5%), tinnitus (63.0%) and head noises (63.0%). The most vulnerable segment of internal jugular vein (IJV) was J3 segment (96.3%). The average styloid process length in our study was 3.7 cm. Hearing impairment was more common in bilateral IJVS (68.8% vs. 18.2%, P=0.018). One patient reported significant relief of symptoms at 1 year follow-up after underwent styloidectomy combined with stenting. Conclusions: Neurological symptoms of eagle syndrome induced IJVS were various, including either arterial or venous issues. Better understanding of this disease entity may be helpful for clinical diagnosis and treatment.