We present here a patient suffering from an unilateral retinal arteriovenous communication, belonging to group 2 in Archer classification, associated to a homolateral vascular malformation into the ophthalmic artery area, and a full-thickness macular hole. This is the first case with such an association reported in the literature.

Although typical FKS would present with optic nerve atrophy and contralateral optic nerve edema secondary to an intracranial mass, in this case, edema was replaced by a hemiretinal vein occlusion. In optic atrophy that does not show characteristic visual field alterations, typical symptoms, or the classic evolution of a given disease, diagnostic imaging may reveal the etiology. As FKS is uncommon, when there is optic atrophy associated with acute pathology of the other eye, a space-occupying lesion should be suspected.