We report the clinical course of cytomegalovirus (CMV) retinitis associated with acquired immunodeficiency syndrome (AIDS) from the initial onset. The patient was a 40-year-old human immunodeficiency virus antibody-positive male with hemophilia A. He was diagnosed as having AIDS on the basis of pneumocystis carinii pneumonia. Ophthalmoscopic examination disclosed a small white punctate lesion at the macular area in his right eye. Because the lesion enlarged gradually with hemorrhages, it was suspected to be CMV retinitis. However, further examination was impossible due to his severe general condition. He died five months later and the autopsy disclosed disseminated CMV infection. Ocular histopathological examination revealed CMV retinitis. The earliest sign of CMV retinitis is supposed to be a white punctate retinal lesion, which becomes a small white patchy lesion resembling a cotton-wool spot. It may gradually progress to diffuse retinal involvement, frequently associated with hemorrhages.