Deferoxamine is a commonly used chelating agent for secondary hemochromatosis. We report a rare retinal manifestation of deferoxamine toxicity in a 68-year-old man and provide supporting multimodal imaging and electrophysiology. The patient had iron overload related to transfusion-dependent myelodysplastic syndrome and developed a pseudovitelliform macular lesion related to deferoxamine toxicity. We also describe for the first time the worsening of this maculopathy on deferasirox, an alternative chelating agent. Macular pseudovitelliform lesion is a unique manifestation of deferoxamine toxicity that can be mistaken for pattern dystrophy. It is important to recognize this manifestation, because discontinuation of the offending agent may halt or reverse the toxicity.