Over the past 20 years, researchers have discovered over 30 separate visual areas in the cortex of the macaque monkey that exhibit specific responses to visual and environmental stimuli. Many of these areas are homologous to regions of the human visual cortex, and numerous syndromes involving these areas are described in the neurologic and ophthalmic literature. The focus of this review is the anatomy and physiology of these higher cortical visual areas, with special emphasis on their relevance to syndromes in humans. The early visual system processes information primarily by way of two separate systems: parvocellular and magnocellular. Thus, even at this early stage, visual information is functionally segregated. We will trace this segregation to downstream areas involved in increasingly complex visual processing and discuss the results of lesions in these areas in humans. An understanding of these areas is important, as many of these patients will first seek the attention of the ophthalmologist, often with vague, poorly defined complaints that may be difficult to specifically define.