We report three cases and a literature review describing a syndrome of intermittent idiopathic focal or segmental dystonic tremor dramatically responsive to clonazepam. All patients were young men who had intermittent symptoms. After magnetic resonance imaging of the brain and laboratory analysis of blood, each patient was treated with clonazepam and clinically observed for 1 year. In all three cases clonazepam produced full abatement of the tremor. None of the patients displayed progression of symptoms, and all have remained tremor free with stable doses of clonazepam. Clonazepam-sensitive intermittent dystonic tremor may represent a benign syndrome occurring in young men.