Essential tremor (ET) is a progressive and highly prevalent neurologic disease. Along with the tremors, mild to moderate gait ataxia and other signs of cerebellar dysfunction may occur (i.e., subtle saccadic eye movement abnormalities and abnormalities of motor timing) as well as cognitive features, some of which may be due to cerebellar dysfunction. Numerous neuroimaging studies indicate the presence of functional, metabolic, and structural abnormalities in the cerebellum of a patient with ET. In tandem with these clinical and imaging studies, which were gathering increasing support for the notion that the cerebellum and/or cerebellar systems seemed to be at the root of ET, a growing postmortem literature is for the first time beginning to identify microscopic abnormalities in the ET brain, most of which are centered on the Purkinje cells and connected neuronal populations, and are likely to be degenerative. In terms of treatment, most of these pharmacotherapeutic agents serve to enhance GABAergic neurotransmission, further bolstering the notion that ET may very well be a disorder with a primary Purkinje cell dysfunction resulting in reduced cerebellar cortical inhibition. Similarly, the interruption of presumably abnormal cerebellar outflow pathways to the thalamus is the mechanism of deep-brain stimulation surgery, which is highly effective in treating ET.