Myoclonic seizures are sudden, brief, shock-like contractions that can vary in distribution and intensity. They may be present in different epilepsy syndromes, including some idiopathic generalized epilepsy, epileptic encephalopathies and progressive myoclonus epilepsies. Despite the fact that there are many studies about the pathophysiology of myoclonic seizures and clear descriptions of the different myoclonic epilepsy syndromes, relatively little has been written on treatment. Valproate and some benzodiazepines are widely used to treat myoclonic seizures. In addition, more treatment options exist today as there is emerging evidence to support the efficacy of some newer antiepileptic drugs. On the other hand, some myoclonic epilepsies remain refractory to drug treatment and some antiepileptic drugs may exacerbate or even induce myoclonus. In the coming years, better understanding of mechanisms of myoclonic seizures and myoclonic epilepsies could result in great improvement of therapy and the quality of life of patients.