A 42-year-old man who was human immunodeficiency virus (HIV)-positive complained of floaters in his right eye, which had existed for 1 week, followed by loss of central vision. Results of ophthalmoscopic examination disclosed confluent yellowish-white retinochoroidal infiltrates with perivascular sheathing, which were more prominent superiorly in the right eye. Approximately 10 small, white, intraretinal and choroidal lesions were observed in the nasal periphery of the left eye. Results of cytologic examination of the vitreous of the right eye showed neoplastic cells characteristic of large cell type malignant lymphoma. Shortly thereafter, cytologic examination of the cerebrospinal fluid also showed large cell malignant lymphoma. Magnetic resonance imaging (MRI) showed a mass involving the left cerebellar hemisphere. After bilateral whole-eye radiation therapy, there was complete resolution of the lymphomatous retinochoroidal infiltrates in both eyes. The ophthalmologic and neurologic manifestations of acquired immune deficiency syndrome (AIDS) are discussed. The authors believe this is the first report of ocular malignant lymphoma occurring in a patient with AIDS.