Macular telangiectasia type 2 (MacTel type 2) is a degenerative disease that affects the retinal pigment epithelium, Müller and glial cells, resulting in vascular changes and photoreceptor loss [1]. It usually presents in middle-aged individuals with characteristic changes in the macular capillary network and atrophy of the neurosensory retina. Clinical manifestations include loss of retinal transparency (“retinal graying”), crystalline deposits in the inner retina, intraretinal pigmentary changes, presence of right angle vessels, accumulation of yellowish material in the central fovea, proliferation of subretinal vessels and late development of choroidal neovascularization [2]. MacTel type 2 is classically considered a bilateral disease with asymmetric or sequential involvement. However, some unilateral cases have been described, with no change in the fellow eye after five or six years of follow-up [3], [4].

A case of MacTel type 2 with unilateral involvement is reported. No changes were observed in the fellow eye after three years of follow-up.