ABSTRACT
We report a case of a 20‑year‑old female having systemic hypertension who presented with right‑sided proptosis, chemosis, and diminished vision, preceded by an acute episode of unilateral throbbing headache. Imaging studies revealed a right‑sided direct, spontaneous carotid‑cavernous fistula (CCF), aneurysm of internal carotid artery, bleed in the parieto‑frontal lobe, and swelling of extraocular muscles. Abdominal ultrasound revealed a small contracted right kidney measuring 64 mm × 27 mm. A direct spontaneous CCF can occur spontaneously following rupture of intracranial aneurysm without any history of trauma or connective tissue disorder. Prompt diagnosis of intravascular malformations at initial presentation can prevent neurological complications and vision loss. A team approach including emergency physicians, neurosurgeons, and ophthalmologists is needed for the proper management of such patients.