Dear Editor Primary intraocular lymphoma (PIOL), a subset of primary central nervous system lymphoma (PCNSL), is the commonest neoplastic masquerade syndrome, often leading to delay in diagnosis and poor prognosis [1]. We report a case of bilateral central retinal artery occlusion (CRAO) preceding ocular recurrence of PCNSL. A 58-year-old male had a left frontal lobe tumour debulked in September 2007. Histology showed a diffuse large B-cell lymphoma in keeping with PCNSL. Staging eye examination at the time was negative. In spite of methotrexate-based chemotherapy, the CNS lymphoma progressed, requiring total brain radiotherapy. Further scanning in February 2008 showed complete response to treatment. His concurrent medical problems included steroid-induced diabetes, hypertension and pulmonary embolism for which he was on low molecular weight heparin. He presented to the ophthalmic department with a 4-week history of right-sided painless visual loss in March 2008. Eye examination revealed no perception of light in the right eye, afferent pupillary defect, pale oedematous retina, a cherry red spot, and segmentation of blood within the retinal vessels caused by CRAO. Left visual acuity was 6/60; fundoscopy revealed background diabetic retinopathy, and age-related macular degeneration. History, clinical examination, and investigations were negative for giant cell arteritis (normal erythrocyte sedimentation rate, C-reactive protein, and blood count). The patient returned 1 month later with painful right rubeotic glaucoma, hyphaema, and vitreous haemorrhage restricting fundoscopy. B-scan ultrasonography revealed a highly reflective choroidal mass, measuring 15.9 × 18.1 mm and 4 mm in height, associated with retinal detachment (Fig. 1). Lymphomatous infiltration was suspected. A substantial vitreous biopsy was negative. Staging magnetic resonance imaging excluded intracerebral recurrence, optic nerve/sheath, and systemic involvement. Despite topical treatment the right eye remained painful and was enucleated. Histopathological examination revealed a necrotic tumour with viable peripheral rim, with sheets of lymphoid cells replacing the retina and confined to the subretinal space. Immunohistochemical staining showed the tumor cells were positive for CD20, MUM-1, BCL-2 with patchy staining for CD30, and were negative for BCL6 and CD10.The proliferation fraction with Ki-67 was high (95%). This confirmed diffuse large B-cell lymphoma identical to the original brain biopsy. A thrombus was identified within a central vessel, in keeping with CRAO. The retina was necrotic, with no vascular invasion. The optic nerve was atrophic, and free from tumour infiltration. Left vision subsequently decreased to perception of light in September 2008. Examination confirmed CRAO. Two weeks later, the retinal oedema decreased and pale retinal creamy lesions with retinal exudation and vitritis became evident, suggesting left ocular lymphoma. Further staging and lumbar puncture confirmed significant recurrent CNS lymphoma, but no evidence of optic nerve/sheath infiltration. A course of palliative left orbital irradiation was completed in November 2008. Unfortunately, the patient died in January. Declaration of interest The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.