Myoclonus is a hyperkinetic movement disorder characterized by quick, involuntary jerks. It encompasses a vast range of etiologies and widespread anatomic locations. Treatment frequently requires multiple agents and is often only partially beneficial. These patients pose a considerable challenge for the clinician, further complicated by the fact that many of the treatment choices lack evidence-based support. In the past few years, publications regarding therapy have been largely observational case reports or series. Although the literature on treatment of cortical myoclonus appears to be growing, evidence regarding myoclonus of noncortical origin is less well established. Investigation of more satisfactory treatments is needed, as this condition can be disturbing, debilitating, and sometimes harmful for patients. Continuing investigations are using various animal models (mostly of posthypoxic myoclonus), electrophysiologic studies, new imaging techniques such as diffusion tensor imaging, and genetic studies. Meanwhile, the clinical approach to diagnosing and classifying myoclonus remains largely unchanged. This review updates readers on current investigations and suggests guidelines for diagnosing and treating myoclonus.