Gilles de la Tourette syndrome is a common multifaceted neuropsychiatric disorder typically commencing in childhood or adolescence. Its pathophysiology is still unclear. The basal ganglia and cortico-striato-thalamo-cortical circuits seem to play an important role. One of the most consistent alterations in children is a volume reduction of the striatum. Additionally, changes have been documented within the white matter of cortico-striato-thalamo-cortical circuit. On a cortical level, alterations have been shown in different regions including the sensorimotor and prefrontal cortex, the supplementary motor area and the inferior parietal cortex (BA 40). Biochemically, an increase in dopamine activity seems to be crucial. Also, serotonin, particularly its dopamine-inhibiting effect is considered to be important. Cognitive-psychological and perceptual-psychological approaches initially focused on an impairment of inhibitory control in Tourette patients. However, different studies yielded inconsistent results. Currently, altered cognitive control in Tourette syndrome is viewed in the context of interference with compensatory mechanisms for tic control. Additionally, alterations of interoceptive awareness have been shown. However, it remains unclear whether they are causative or represent secondary compensatory mechanisms. One of the most recent approaches is based on the Theory of Event Coding based on the concept that both perception and action are stored together in so called event files. Thus, perception and action are tightly connected, which is thought to be even stronger in Gilles de la Tourette Syndromes patients compared to healthy controls resulting in tics being triggered by various stimuli being linked to them.