The purpose of our study was to define the neuroimaging features of the cardiolipin antibody syndrome. Thirty-eight patients with elevated anticardiolipin antibody titers were studied with magnetic resonance imaging or computed tomography or both. Two patients underwent cerebral angiography. All patients had recurrent transient ischemic attacks, amaurosis, or strokes. One patient had normal imaging findings. The remaining patients had a combination of infarction and atrophy. Focal infarcts, the most common finding, were seen in 32 patients. Cerebral atrophy was seen in 26 patients and was the only radiographic finding in 5. Angiography demonstrated dramatic abnormalities in the distal portions of the anterior and posterior circulations, with multiple stenosis and occlusions and extensive pial and transdural collateral networks. The cardiolipin antibody syndrome should be suspected in young patients with transient ischemic attacks or strokes in the absence of the usual risk factors for cerebrovascular disease. The presence of raised anticardiolipin antibody titers or the cardiolipin antibody syndrome in patients with lupus, in those with other connective tissue diseases, and in patients without overt manifestations of an autoimmune disorder should be viewed as a risk factor for future ischemic cerebrovascular events. Further understanding of the precise role of these antibodies in the pathogenesis of vascular thrombosis may lead to a better understanding of the mechanisms underlying certain forms of stroke.