Essential tremor (ET) is considered to be a monosymptomatic disorder consisting primarily of postural hand tremor. Nevertheless, clinical expression can vary based on the body region affected by tremor and the coexistence of other neurologic signs, such as tandem gait disturbance. We conducted a two-part study to test the hypothesis that variability in ET clinical expression is influenced by gender and age. In part 1, we examined a large ET clinical database (n = 450), comparing ratings of postural hand and head/voice tremor based on gender. Head/voice tremor was significantly more frequent and more severe among female ET patients; men had more severe postural hand tremor. In part 2, ET patients (n = 40) had significantly more missteps when tandem walking in comparison to age-matched controls. Poor tandem walk in ET cases was associated with more advanced age, but not gender, disease duration, or ratings of postural hand or head/voice tremor. We conclude that gender influences the body region most affected by ET possibly through the effects of the sex chromosomes or hormones. Ataxia (tandem gait difficulty) is common in ET and may be an accentuation of cerebellar dysfunction due to aging.