The Tourette syndrome (TS) represents an intriguing disorder that overlaps the clinical fields of neurology and psychiatry. Composed of a spectrum of familial, involuntary motor and vocal tics and co-morbid neuropsychological problems, this disorder has become a model for investigators interested in neurobehavioral research. Although the subject of numerous reports since Tourette's original description in 1885, over the past decade there has been a rapid expansion of knowledge pertaining to the phenomenology of tic syndromes and their associated problems, the role of genetic and environmental factors, and the underlying pathophysiology. The role of frontal-subcortical circuits, dopaminergic neurotransmission, and second messenger systems are highlighted. This report provides a framework for understanding current neurobiological issues in TS.