We described a case of a 29 year-old heterosexual man with a history of blurry vision in the left eye followed by complete blindness, who was referred to our hospital because of prolonged fever and chills. The patient was diagnosed with AIDS and an analysis of paracentesis fluid from the left eye by polymerase chain reaction (PCR) revealed Mycobacteria avium (M. avium) infection. There have been many reports of ocular infection of M. avium found on autopsy in AIDS patients, but only one case has been reported in the eye of a living AIDS patient. This paper will be the second antemortem case of M. avium infection in the eye of an AIDS patient, in which the eye manifestation was the first symptom for AIDS.