The jugular Eagle syndrome represents the last described variant of the Eagle syndrome, in association with chronic cerebrospinal venous insufficiency; it is characterized by the internal jugular vein (IJV) compression between the transverse process of C1 and an elongated and angulated styloid bone. Headache, numbness and dizziness are the main symptoms reported by patients. Moreover, the IJV compression may exacerbate intracranial hypertension and neuroinflammation (due to brain catabolite stasis) and contribute to develop impairment of working memory, chronic fatigue and nonaneurysmal subarachnoid and perimesencephalic haemorrhage, even if a clear pathogenetic mechanism is still unknown. Doppler ultrasound scan and second-level imaging, through MRI and CT, are fundamental to characterize and plan an eventual surgical treatment through styloidectomy or C1 lateral mass resection. Further studies to characterize this new entity and its impact on brain circulation and neurological disease development are warranted.