An unusual case of CMV retinitis and AIDS who developed immune recovery uveitis (IRU) despite a low CD4+ T cell count achieved during HAART is reported. A 36-year-old female complained of blurred vision in both eyes six months after initiation of anti-CMV retinitis therapy and HAART. Ocular examination revealed a substantial intraocular inflammation causing a dense vitreous haze and frosted branch angiitis. Consecutive CD4+ T cell counts were 20 cells/mm(3) or less and plasma HIV mRNA was undetectable. The laboratory test for Cytomegalovirus was positive whereas those for infections known to cause uveitis were negative. The inflammatory reaction resolved with treatment, but she developed retinal detachment just before she died of pulmonary complications. A review of the literature led us to propose that our patient developed an intraocular inflammation which may probably be a form of IRU and it might be appropriate to employ additional criteria in the definition and the diagnosis of IRU.