Tourette syndrome is a hyperkinetic movement disorder that presents before age 18 years and involves motor and phonic tics that may present with a wide range of severity. The severity and presentation of tics in an individual may fluctuate over time. Tourette syndrome may affect social relationships and school attendance, and may result in depression. Comorbidities are common, with attention-deficit/hyperactivity disorder and obsessive-compulsive disorder being most common. The literature supporting optimal treatment is limited but provides a framework for clinical decision-making. The focus of this review is to discuss the symptoms and possible causes of Tourette syndrome and current non-pharmacologic and pharmacologic treatment options, to help practitioners optimize care for pediatric patients with this disease.