Isolated cilioretinal artery occlusion is uncommon but has characteristic features. Based on a case report and a review of the literature, we present the clinical findings and angiographic particularities of this syndrome and discuss controversial pathophysiological data.A 56-year-old man had sudden visual loss in the right eye estimated at 3/10 P10. Fundus examination showed areas of retinal interpapillomacular infarction due to cilioretinal artery occlusion. Fluorescein angiography demonstrated delayed filling and emptying of this artery. Left fundus examination was normal.Systemic examinations revealed severe hypertension (240/130) rapidly controlled with a two-drug regimen. The clinical course was good with normalization of fundus and angiography, and visual recovery to 8/10 P3 with a small absolute paracentral scotoma.The few cases described would offer an explanation of the low prevalence of cilioretinal arteries and the more frequent association with central retina venous obstruction which can mask arterial occlusion. A relative reversible occlusion explains the generally good prognosis especially if the capillary network is not affected by the occluded artery as was observed in our case.Although diagnosis of isolated cilioretinal artery occlusion is made without difficulty, the underlying pathogenic mechanism remains difficult to explain due to the various phenomena revealed by the increased arterial pressure.