Purpose The aim of this study is to highlight findings of retrograde maculopathy (RM) on optical coherence tomography (OCT) as a sequela of neuromyelitis optica associated optic neuropathy. Observations We report two patients with demyelinating lesions involving the visual pathways due to neuromyelitis optica in whom RM was seen, and in whom the location of the RM corresponded to their visual deficits. In one case, we describe a patient who had a demyelinating chiasmal lesion with bitemporal hemianopia and who had corresponding binasal RM. In another case, we describe a patient who had generalized vision loss in the right eye and who had corresponding diffuse RM in the right macula. Conclusions and Importance RM is a potential sequela of severe optic neuropathy in neuromyelitis optica and topographically corresponds to severe visual field defects, as well as severe ganglion cell complex loss. Providers should recognize this finding and be aware that they do not need to pursue diagnostic evaluation due to concern for primary macular disease.