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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. Sp
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