The traditional view on cerebellar functioning has recently been challenged by results from neuroanatomical, neuroimaging and clinical studies. In this contribution, eighteen patients with primary cerebellar lesions (vascular: n =13; neoplastic: n =5) were systematically investigated by means of an extensive neuropsychological test battery. Fifteen patients (83%) presented with a broad variety of cognitive and linguistic deficits following cerebellar damage. Disturbances of attention (72%), executive functioning (50%) and memory (50%) were most commonly found. Analyses of our results tend to support the hypothesis of a lateralization of cognitive modulation within the cerebellum, the right cerebellar hemisphere being associated with logical reasoning and language processing and the left cerebellum mediating right-hemispheric functions including attentional and visuo-spatial skills. In addition, nine patients (50%) presented with frontal-like behavioural and affective alterations. In an attempt to determine the working-mechanism underlying cerebellar-induced cognitive and affective disturbances, all patients were investigated by means of quantified Tc-99m-ethylenecysteine dimer (ECD) single photon emission computerized tomography (SPECT) studies. From a semiological point of view, damage to the cerebellum can cause a broad spectrum of clinically significant cognitive and affective disturbances. From a pathophysiological point of view, quantified SPECT data, reflecting the phenomenon of cerebello-cerebral diaschisis, support the functional impact of the cerebellar lesion on cortical functioning through disruption of cerebello-cerebral connections.