Recent advances in medications and surgical therapy for neurological disorders may offer new therapeutic options for the treatment of myoclonus. Appropriate therapy for myoclonus depends on the etiology, and in some cases, myoclonus can improve when the provoking cause is eliminated. When the underlying cause for the movements is not immediately reversible, localization, disease pathophysiology, and etiology may each play a role in determining the most appropriate symptomatic treatment of disabling myoclonic jerks. While the use of many agents is still based on small, open-label case series and anecdotes, there is a growing body of evidence from head-to-head comparative trials in several types of myoclonus that may help guide therapy. New therapies for refractory myoclonus, including sodium oxybate and even deep brain stimulation, are also being explored with increasing enthusiasm.