Pulmonary arteriovenous fistula (PAVF) is a rare condition which occasionally causes neurological complications. A 43-year-old female with multiple PAVFs presented with several episodes of amaurosis fugax and transient right hemiparesis. She had no other vascular abnormality, and her human leukocyte antigen haplotype did not coincide with previous patients with hereditary hemorrhagic telangiectasia. She underwent PAVF embolization to prevent further neurological complications, and had an uneventful subsequent clinical course. Amaurosis fugax is a slight neurological symptom and may be an early important sign of PAVF. We stress that PAVFs should be considered in the differential diagnosis of patients with amaurosis fugax who complain of exertional dyspnea or demonstrate cyanosis.