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There are some unusual neuropsychological syndromes rarely reported in neuropsychological literature. This article presents a review of four of these unusual clinical syndromes: (a) Central achromatopsia (loss in the perception of colors due to a lesion of the visual association cortex); (b) Bálint’s syndrome (simultanagnosia, optic ataxia and gaze apraxia associated with bilateral parietal-occipital pathology); (c) pure word-deafness (clinical syndrome characterized by an inability to understand spoken language with preserved speech production and reading ability); and (d) aphasia of the supplementary motor area (significant difficulty in initiating and maintaining speech, regardless if the patient makes significant effort to speak due to a damage in the left supplementary motor area). It is concluded that although these syndromes are rare, it is fundamental to consider them to understand the brain organization of cognition. Their understanding is also crucial in the clinical analysis of patients with brain pathologies.