Purpose <p>This case report aims to describe an atypical presentation of pigmented paravenous chorioretinopathy (PPCRA).</p> Methods <p>Detailed clinical ophthalmologic examinations, multimodal imaging and electroretinography of a 33-year-old woman who presented with unilateral PPCRA.</p> Results <p>A 33-year-old female who is known to have hypothyroidism and had previous bariatric surgery, referred for retinal evaluation following incidental findings during a refractive surgery consultation. Fundus examination revealed unilateral segmental perivascular hyperpigmentation, vascular sclerosis, and areas of chorioretinal atrophy, raising the differential diagnosis of pigmented paravenous chorioretinopathy (PPCRA) versus resolved retinal vasculitis. The patient reported no significant ocular symptoms apart from decreased night vision in one eye and denied a history of acute visual loss or photophobia. Systemic workup, including autoimmune and infectious serologies, imaging, and a detailed clinical history, was unremarkable. The patient reported consanguinity within the family.</p> Conclusion <p>This report underscores the challenge of distinguishing PPCRA, a rare, typically bilateral hereditary condition, from resolved vasculitis, which often presents unilaterally with a history of systemic inflammation. Fluorescein angiography and optical coherence tomography were instrumental in identifying the lack of active inflammation and vascular leakage, favoring the diagnosis of PPCRA.</p>