Purpose: To present a rare manifestation of macular telangiectasia type 2 (MacTel type 2) followed up for over six years. Methods: A 61-year-old woman with one year history of blurred vision of her left eye was referred. Results: Whereas the funduscopy, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and fundus autofluorescence (FAF) were normal in the right eye, they revealed noticeable findings typical of MacTel type 2 in the left eye. After over six years follow-up, OCT-angiography (OCTA) showed no remarkable difference between the two eyes, and en face OCT showed subtle abnormal change in the right eye as well as typical pathological changes in the left eye. Conclusion: MacTel type 2 can present unilaterally and remain so for a long time. The role of multimodal imaging in diagnosis and follow-up is of utmost importance. Keywords: Unilateral, Macular telangiectasia type 2, Multimodal imagin