This chapter describes the clinical phenomenology and pathophysiology of Gilles de la Tourette syndrome (GTS) and reviews current structural and functional neuroimaging data of this fascinating neuropsychiatric condition. Significant neuroimaging evidence exists for a primary cortical dysfunction in GTS. Structural changes were reported in the basal ganglia, including both the striatum and the globus pallidus. Diffusion tensor imaging (DTI) has pointed to the microstructural abnormalities in white matter in GTS patients, including the corpus callosum and anterior and posterior limb of the internal capsule. Defects in brain maturation could be one of the pathophysiological mechanisms that lead to emergence of the GTS symptoms in childhood and their persistence into adulthood. Evidence from pharmacological trials, especially the fact that dopamine receptor blockers are the most effective treatment for tics to date, and postmortem analyses suggested that abnormalities of dopaminergic neurotransmission play a key role in the pathogenesis of GTS.