Vasospasm has been discussed as a less frequent cause of amaurosis fugax. Since its direct demonstration is difficult, its diagnosis is usually based on the exclusion of other causes and/or response to calcium entry blockers. We describe diagnosis and successful treatment of vasospastic amaurosis fugax in a patient with systemic autoimmune disease: A 54 year-old patient with an overlap collagenosis presented with relapsing episodes of transient monocular blindness. Angiography and transcranial Doppler scanning revealed a high-grade stenosis of the left ophthalmic artery. After administration of oral nimodipine the attacks ceased immediately and repeated Doppler examinations confirmed resolution of the stenosis. We infer that vasospasm of inflammatory altered cerebral vessels may contribute to focal neurological deficits in patients with systemic autoimmune disease. Calcium entry blockers should be discussed as a possible treatment in patients with systemic autoimmune disease and evidence of functional disturbances of cerebral blood flow.