Gilles de la Tourette syndrome (GTS) is a chronic tic disorder characterised by the presence of multiple motor and vocal tics with onset during development. Tics are the most common hyperkinetic symptoms in childhood and co-morbid behavioural conditions (especially obsessive-compulsive disorder, attention-deficit and hyperactivity disorder, affective symptoms, and impulsivity) are present in the majority of patients. Although GTS is no longer considered a rare medical curiosity, its exact pathophysiology remains elusive. Recent research on the brain correlates of the subjective ‘urge to tic’ has highlighted the role of extra-motor pathways within the brain mechanisms of tic generation. Advances in our understanding of the pathophysiology of GTS can pave the way to the implementation of more effective treatment strategies for this heterogeneous neurobehavioral condition. Finally, the development of GTS-specific instruments for the assessment of health-related quality of life has allowed more standardised assessments across the lifespan, capturing the impact of both tics and behavioural co-morbidities.