An 80-year-old right-handed man suddenly became impossible to recognize any color 7 years prior to admission. He complained that everything looked like of the same color, monochromatic. On admission, he could not discriminate any color and any familial face. Left homonymous hemianopsia associated with right lower partial visual filed defect was observed, but visual acuity of both eyes was well preserved. Visual-visual color tasks (Ishihara, matching, Hue test, Panel-D15) disclosed the disturbances in color perception. However, he could roughly distinguished red or brown from the other colors. The color test was also impaired regarding the visual-verbal color tasks (naming, pointing). However, verbal description of the color concept, which was shown by the verbal-verbal color tasks, was well preserved. In addition, we observed left hemispatial neglect, disturbance of face recognition and topographical disorientation. MRI revealed old hemorrhagic infarcts in the bilateral occipital and temporal lobes, including the bilateral lateral and medial occipito-temporal gyri. Disturbance of color recognition in this case was diagnosed as cerebral achromatopsia on the basis of clinical characteristics and MRI findings. This is the first case of cerebral achromatopsia of which lesions were detected by MRI in detail.