To describe a case of vitreoretinal lymphoma with unilateral vascular involvement. A 80-year-old male with recurrent amaurosis fugax (AF) in the left eye was seen in the ophthalmological department. During a period of seven months the patient developed central vein occlusion (CRVO) and later the panuveitis suspected to be acute retinal necrosis (ARN) before being diagnosed with vitreoretinal lymphoma (VRL). The patient preferred enucleation as treatment which allowed thorough histological examination revealing Epstein Barr virus-associated vitreoretinal lymphoma. Vascular involvement with AF and CRVO is yet to be described in relation to VRL. This case-study underlines the critical importance of considering VRL when the clinical course deviates from the expected.