Ocular lymphoma involvement can be either secondary during systemic lymphoma or primary.

Diagnosis can be troublesome due to insidious disease onset. Uveitis is the main differential diagnosis.

The prognosis is poor.

A 62-year-old male patient was evaluated during a periodical check-up for hypertensive retinopathy. The unexpected good vision quality with severe left vitreous infiltration and not associated macular edema contributed to malignancy suspicion. A diagnostic procedure was performed bilaterally. Both of the vitreal tissue revealed atypical lymphoid cells with B-Cell phenotype. Cranial MRI, PET-CT, and CSF analysis documented the case as primary vitreoretinal lymphoma (VRL).

First-line treatment was with intravitreal methotrexate (MTX). After 10 courses, high-dose cytarabine-based treatment was given as consolidation. Considering high recurrence rates, stem cells were mobilized and cryopreserved for future use for autologous stem cell transplantation (ASCT).

Follow-up was 3 monthly. After 10 months of remission period, retinal disease relapse was spotted. After 5 cycles bilateral intravitreal MTX, disease progressed as leukemic invasion of left optic nerve. High dose chemotherapy followed by ASCT was performed.

Diagnosis of IVL is challenging due to late onset macular edema. Related with high relapse rates withhigh mortality, high-dose chemotherapy is the recommended management type currently.