We examined ten patients from a consecutive series of 73 patients with either isolated cytomegalovirus papillitis or limited cytomegalovirus retinitis contiguous with the optic disk. Patients with peripheral retinitis and other areas of retinitis were excluded. All patients were treated with ganciclovir. Two distinct types of cytomegalovirus infection of the peripapillary area were identified. Type I was characterized by spread of limited retinitis to the optic disk margin, good central visual acuity, and permanent arcuate and altitudinal visual field defects that enlarged and became more complete as the retinitis progressed toward the disk. Type II appeared to be a true cytomegalovirus infection of the optic nerve characterized by primary, isolated papillitis with peripapillary retinitis, an early afferent pupillary defect, and good initial visual acuity, which rapidly deteriorated despite prompt antiviral therapy. Peripapillary cytomegalovirus retinitis appears to be an important and underreported cause of visual morbidity in patients with AIDS.