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One in 20 essential tremor (ET) cases arises during childhood. We report 19 pediatric ET cases (mean age = 12.7 years). The majority (68.4%) were male, and only one had head tremor. Childhood and adult forms of ET may differ in several important respects, providing information about the underlying biology of ET. A possible male preponderance in childhood ET cases could reflect a modification of disease expression by gender, such that males manifest the disease at an earlier age than females. A paucity of childhood cases with head tremor suggests that the neuropathological changes in ET may evolve somatotopically. Head tremor may require midline or more extensive bilateral pathology which may only occur later in the disease. © 2001 Movement Disorder Society.