Stylo-carotid Eagle syndrome, also known as vascular or carotid artery syndrome, represents a rarer form of the Eagle syndrome. Epidemiological specific data are not available in the literature, but it mainly affects middle-aged females. In this variant, the compression of an elongated styloid process involves the extracranial cervical carotid axis and the nervous plexus that surrounds it. Clinically, it could be completely asymptomatic and suddenly exhibits with neurovascular complications such as headache, transient visual loss, syncope, pseudoaneurysm formation, and the more life-dangerous transient ischemic attacks (TIAs), stroke, vessel stenosis, dissection, or occlusion. When the styloid compression involves the perivascular nervous fibers, the patient experiences pain in the ipsilateral face and neck regions, exacerbated by rotational head movements. Differently from the classic syndrome, a computed tomography angiography (CTA) is fundamental to evaluate the vascular situation. Treatment is based on removing the cause of the compression, through styloidectomy, but includes the management of the pharmacological therapy (beginning of antiplatelet therapy) and neurovascular complications and the repair through surgical or endovascular approaches of the vessel damage (stenting, aneurysmectomy, etc.). For these reasons, the stylo-carotid syndrome could represent a rarer but dangerous variant of the Eagle syndrome that requires a proper diagnosis and treatment.