Approximately 5% of new essential tremor (ET) cases arise during childhood. The goal of the current report was to examine the possible association between male gender and pediatric ET, using data from 95 pediatric ET cases seen at three medical centers (two in the United States and one in Spain). The odds of ET in our sample of cases were threefold higher in boys compared to girls. Whether this association between male gender and pediatric ET represents a selection bias or a true gender-mediated biological difference in disease expression is not known, although some data support the latter possibility. © 2005 Movement Disorder Society.