A number of studies reported the possible differences between men and women in movement disorders. Evidence shows that estrogens may have a neuroprotective effect and may modulate the neurodevelopment of the different brain structures. Movement disorders including Parkinson's disease, dementia with Lewy body, Huntington's disease, Tourette's syndrome, and dystonia among others display significant clinical differences between sexes, with structural differences in the dopaminergic pathways between men and women. Here we summarize the most relevant clinical aspects of some of the most common movement disorders, highlighting the differences in disease onset, clinical presentation, therapy, and outcomes. Increased recognition of these differences may help physicians better understand the pathophysiology of these conditions and provide a tailored therapeutic approach.