A case of primary intraocular malignant lymphoma without cerebral involvement is reported in a 30-year-old man with acquired immunodeficiency syndrome. The study of the enucleation specimen showed a B immunoblastic lymphoma with a CD30 positive anaplastic large cell component. There was no involvement of the adnexal structures of the orbit. The patient subsequently completed non-surgical staging showing no extension of the tumour. The clinical course was rapidly fatal with dissemination to the pericardium and pleura.