Purpose <p>Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare, poorly understood chorioretinal disorder characterised by perivenous atrophy and pigment clumping. This systematic review aimed to synthesise the current evidence on its clinical features, diagnostic modalities, and management strategies, while identifying knowledge gaps and opportunities for future consensus guidelines.</p> Methods <p>A systematic review was conducted following PRISMA guidelines and registered in PROSPERO (CRD42022346753). Nine databases were searched for English-language publications between January 1980 and April 2025. Eligible studies included case reports, case series, reviews, and commentaries reporting clinical, diagnostic, or therapeutic data on PPRCA. Data were extracted by independent reviewers, risk of bias was assessed with the CASP checklist, and findings were synthesised thematically and descriptively.</p> Results <p>Seventy-two studies reporting on 162 patients were included. The mean age was 38 years, with a slight female predominance (54%). Ophthalmoscopic findings—most notably chorioretinal atrophy (42%) and pigment clumping (35%)—were the most frequently described features. Imaging techniques such as ultra-wide field fundus photography and fluorescein angiography were widely used but lacked diagnostic uniformity. Electroretinography revealed variable functional deficits. Management was largely supportive, targeting associated complications (e.g., macular oedema, glaucoma), with limited long-term follow-up data.</p> Conclusion <p>PPRCA remains a clinically heterogeneous and under-recognised condition without standardised diagnostic or therapeutic protocols. This review highlights the need for consensus diagnostic criteria, longitudinal registries, and collaborative studies to improve clinical care and inform future research into its pathogenesis and management. </p>