A 64-year-old Caucasian woman was referred to our hospital after referring vision loss; she had been previously diagnosed with systemic diffuse large B-cell lymphoma. Retinal and optic nerve involvement were found in her right eye, and iris and vitreous involvement were found in her left eye. Vitreoretinal lymphomas (VRL) are rare in medical literature, hence there is a lack of standardised studies regarding therapeutic options in these patients. In our patient, we decided to add intravitreal injections of rituximab and methotrexate to systemic chemotherapy, and to perform pars plana vitrectomy.