Tourette syndrome (TS) is a childhood onset neurodevelopmental disorder characterized by semi-involuntary, repetitive movements and sounds (motor and phonic tics). Transient tics in childhood are common, and their persistence in TS may be due to failure of maturation of frontal-subcortical circuits mediated by genetic predisposition and environmental factors. Tic improvement by young adult years is common, but its mechanism and predictive factors are unclear. Though tics can often be managed with nonmedical therapies, pharmacotherapy is often used for refractory, severe, or injurious tics but is complicated by side effects and incomplete benefit. This review summarizes the current understanding of TS pathophysiology, current and future treatment options, and recommendations for future research.