OBJECTIVE To report a patient with central retinal artery occlusion with double cilioretinal artery sparing. METHODS Retrospective case report. RESULTS A 45-year-old woman presented with sudden vision loss in her left eye for 10 days. Her best-corrected visual acuity was counting fingers at 2 meters in the left eye and 20/20 in the right eye. Funduscopy showed retinal opacity of posterior fundus, most marked in the macular region, and two small areas of the normal retina; one temporal to the optic disk and the other superior to the optic disk corresponding to the patent cilioretinal retinal arteries in the left eye; the right eye was normal. Fundus fluorescein angiography showed rapid filling of the two cilioretinal vessels and delayed filling of the central retinal artery at initial and last visits. Spectral domain optical coherence tomography findings disclosed diffuse thickening of the neurosensory retina in the perifoveolar area except two small areas at the first visit. After 6 months, spectral domain optical coherence tomography revealed diffuse thinning of the neurosensory retina except two small areas of the retina fed from two patent cilioretinal arteries. CONCLUSIONS In this report, we documented double cilioretinal artery sparing in a patient with central retinal artery occlusion. It seems that a second cilioretinal artery can be present in some patients with central retinal artery occlusion.