Primary vitreoretinal lymphoma (PVRL) represents the most prevalent subtype of primary intraocular lymphoma, predominantly exhibiting diffuse large B-cell lymphoma histopathology. This malignancy is characterized by poor prognosis and frequent central nervous system involvement. Current therapeutic strategies for PVRL are diverse, including local chemotherapy, systemic chemotherapy, targeted therapy, and autologous stem cell transplantation, yet marked by significant therapeutic heterogeneity and lack of standardized protocols. In recent years, clinical advancements have been achieved with local therapies (e.g., intravitreal methotrexate and rituximab and systemic treatments (e.g., Bruton's tyrosine kinase inhibitors). However, the absence of standardized treatment protocols remains a significant clinical challenge. Recent years have witnessed notable progress in both local and systemic treatment modalities. Nevertheless, existing evidence is primarily derived from small-scale retrospective studies with inherent limitations, including suboptimal study designs, therapeutic heterogeneity, and insufficient patient stratification. These constraints have hindered the establishment of evidence-based treatment consensus. Future research should focus on conducting multicenter prospective studies, establishing international collaborative networks, and implementing long-term follow-up protocols to facilitate precision medicine approaches. Concurrent exploration of novel therapeutic targets and pharmacological agents is imperative to transform PVRL management from empirical practice to evidence-based personalized medicine, ultimately improving clinical outcomes.