Gilles de la Tourette syndrome is a neurological disorder characterized by the presence of motor and vocal tics. Other associated features include complex motor and vocal tics, obsessive compulsive behavior and attention-deficit disorder. Although initially thought to be a rare condition, it now appears that the gene for Tourette syndrome may be present in as many as one in a thousand people, and is transmitted as an autosomal dominant trait. However, many individuals carrying the gene may exhibit only minimal symptoms. Neuropathophysiology of Tourette syndrome is unknown but investigations have suggested that the cause may be an abnormality either in the dopaminergic or endorphin receptor system within the basal ganglia. Treatment of Tourette syndrome involves education and counseling of the patient and family. Medications such as neuroleptics, serotonin-reuptake inhibitors, and stimulants are available to treat the manifestations of Tourette syndrome and need to be individualized for each patient.