We report a case of amaurosis fugax associated with ipsilateral internal carotid artery agenesis.

A 50-year-old woman presented with amaurosis fugax in her left eye; the frequency of episodes of the condition had recently increased to once a month. She had a history of hypertension and dyslipidemia, and was under medical therapy. The visual acuity of both eyes was 20/20. Slit-lamp examination was normal except for pseudophakia. Ophthalmoscopy revealed a myopic tigroid fundus and a myopic tilted disc. No abnormalities were evident in fluorescein fundus angiography. Brain computed tomography showed that the left bony carotid canal was absent, and magnetic resonance angiography showed that the left internal carotid artery was also absent. She was diagnosed with left internal carotid artery agenesis. Other neurological and hematological parameters were within normal ranges. The amaurosis fugax spontaneously disappeared and has not recurred over the past 12 months. Our case, although rare, suggests that amaurosis fugax may be associated with internal carotid artery agenesis.