Myoclonic epilepsy in the form of status epilepticus is an extremely rare reported presentation. Herein, we describe an 87-year old male patient presenting with abrupt-onset rhythmic myoclonic jerks that were evaluated as synchronous positive and negative myoclonus. Further etiological investigations revealed that the myoclonus was associated with ictogenesis, and appropriate antiepileptic treatment provided total cessation of the movements. To my knowledge, this is a unique case of myoclonic status epilepticus in an elderly patient, which may have been associated with a cerebrovascular disease. In the presentation of this case, we will review the related literature and discuss some considerations to explain the pathophysiology of epileptic myoclonic movements and the possible role of pontine lesions.