An unusual presentation of macular telangiectasia type 2 with a large pigment deposit.

Macular telangiectasia type 2 (MacTel) is a bilateral retinal disease that seems to be limited to the juxtafoveal region of the macula. We herein report an unusual presentation of MacTel with a large pigment deposit at the macula. Fundus of the right eye showed a large pigment deposit at the macula and right-angled venule. The left eye fundus showed a grayish discoloration due to subretinal fibrosis, dark pigment clumps and right-angled venule in the macula. Lesions were highlighted on multicolor imaging and blue reflectance imaging. Spectral domain optical coherence tomography (SD-OCT) of both eyes showed hyperreflectivity on the inner aspect of the retina corresponding to the area of pigment clumping.