Corticobasal syndrome (CBS) is the clinical presentation of corticobasal degeneration (CBD), a rare neurodegenerative disorder, with features of both cerebral and basal ganglia involvement. Visual disturbance is uncommonly a predominant symptom but when present can be markedly debilitating. Visual findings primarily manifest as oculomotor apraxia, but significant cognitive impairment may result in the inability to process visuospatial information and can result in simultagnosia and visuomotor ataxia. A 60-year-old woman with a history of CBS presented with progressive visual impairment. Her symptoms were primarily due to severe oculomotor apraxia, optic ataxia, and pronounced simultagnosia. We present the case and review the literature regarding visual dysfunction in CBS.