The seven patients described where suffering from simultaneous occlusion of the central retinal vein and cilioretinal artery. All underwent complete ocular examination including fluorescein angiography and visual field assessment, and general investigation with special emphasis on blood and vascular disorders. All patients had been observed for at least one year. Their age ranged from 35 to 60 years (mean: 46 years); fundus examination showed a common pattern consisting of mild edematous central retinal vein occlusion with few hemorrhages as well as a large area of retinal infarction due to cilioretinal artery occlusion. Despite the presence of this large infarcted area, this cases were very different from the ischemic forms of central retinal vein occlusion since in all cases perfusion of the retinal capillaries in the cilioretinal area recovered in a few days. In 4 of 7 cases, fluorescein angiography also disclosed sectorial hypoperfusion of the optic disc at the acute stage. Recovery of venous occlusion occurred in 3 to 14 weeks and the visual acuity outcome was fairly good, as in 5 of the 7 patients, visual acuity reached at last 0.5. No embolic disease was detected but 3 patients had severe migraine. The pathogenesis of this condition remains unclear. Although cilioretinal infarction is usually thought to be secondary to central retinal vein occlusion, the possible role of primary occlusion of the cilioretinal artery is considered.