This is a case presentation of paracentral acute middle maculopathy in a 33-year-old white man with borderline hyperlipidemia. Paracentral acute middle maculopathy was originally thought to be a variant of acute macular neuroretinopathy; however, it is now generally accepted that these two conditions are separate disease entities. The etiology, evolution, and pathophysiology of acute macular neuroretinopathy and paracentral acute middle maculopathy are discussed, as well as current diagnostic techniques. A 33-year-old white man with borderline hyperlipidemia presented with an acute, small paracentral scotoma involving the left eye. Clinical examination revealed a small wedge-shaped retinal lesion that corresponded to scotoma. Spectral domain-optical coherence tomography of the lesion demonstrated irregularity in the middle retina, whereas fluorescein angiography showed subtle hypofluorescence of the lesion. Spectral domain-optical coherence tomography was facilitated in monitoring retinal changes as the patient continued to have a persistent scotoma despite resolution of the retinal lesion. Clinically, acute macular neuroretinopathy and paracentral acute middle maculopathy present in very similar fashions with localized scotomas, blurry vision, and subtle retinal lesions that can be difficult to discern funduscopically. At this time, there is no ocular treatment for either condition, but observation and patient education regarding possibly persistent scotomas are necessary. This atypical case highlights paracentral acute middle maculopathy in a relatively young man with a systemic history significant only for borderline hyperlipidemia. Eye care providers should be familiar with acute macular neuroretinopathy and paracentral acute middle maculopathy to enable them to perform appropriate diagnostic testing and to identify patients who require a systemic disease evaluation.