Despite recent advances in diagnosis and treatment, the prognosis of primary intraocular lymphoma (PIOL) remains poor, and the optimal treatment has yet to be defined. This review presents an overview of the current status of PIOL with a focus on recent advances in diagnosis and treatment. Recent studies show a decreasing time interval from symptom presentation to diagnosis, likely due to increased awareness of the disease and improved diagnostic tools. Advances in the pathologic characterization of PIOL have been made over the past years, and the development of PIOL animal models can offer the opportunity to study aspects of PIOL pathology, pathogenesis and treatment. Regarding treatment of PIOL, recent larger case series favor the use of systemic chemotherapy plus local ocular treatment as first-line management for patients with concomitant PIOL and PCNSL. For patients with isolated PIOL, the current trend focuses on local first-line treatment and early diagnosis of PIOL. Future studies are needed to confirm these trends. Future directions in the management of PIOL should include studies about pathogenesis, prognostic factors, and treatment optimization. In the future, monoclonal antibodies and radioimmunotherapy could prove useful for PIOL treatment.