Takayasu disease was first reported in 1908 by Mikito Takayasu as "a case of peculiar changes in the central retinal vessels." Because in these patients the pulse of the radial artery is impalpable, investigations focusing on the ischemic symptoms of the upper body were conducted. In 1948, Shimizu and Sano named this pathological condition "pulseless disease." Since then, the lesions of Takayasu disease have been detected not only in the aortic arch and its main branches but also in various vessels, including the abdominal aorta and renal arteries. The ocular symptoms of Takayasu disease are considered to be due to ischemia in the retina and choroid. The typical wreath-like arteriovenous anastomosis around the disc reported by Takayasu is observed at a relatively late stage of the disease. The characteristic fundus findings of Takayasu disease include tortuosity and dilatation of the central retinal artery and vein, retinal arteriovenous anastomosis, prominent retinal vasculature, microaneurysms in the capillaries, occlusion of retinal arterioles, soft exudate, choked disc, and optic atrophy. Fluorescein angiography reveals retinal microaneurysms, sludging, slower blood flow, dilatation of retinal vessels, leakage of fluorescence dye due to increased vascular permeability, and arteriovenous anastomosis. Arteriovenous anastomosis initially appears in the periphery at the early stage, and in the arteriovenous crossing at the advanced stage. Systemic administration of corticosteroids is required to prevent vascular stenosis during the early stages of Takayasu disease. Reconstruction of the carotid artery may improve subjective symptoms and fundus findings.