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To describe a potential case of pentosan polysulfate maculopathy that appeared to manifest nearly three years after drug cessation. Complete ophthalmic examination including multimodal fundus imaging, electroretinography, automated perimetry, and molecular testing were performed. A 44-year-old female with a 435g cumulative exposure to pentosan polysulfate sodium presented 38-months after drug cessation with six months of worsening metamorphopsia and prolonged dark adaptation. Fundus exam and multimodal fundus imaging demonstrated charact