Juvenile myoclonic epilepsy is one of the most common epilepsy syndromes, which accounts for nearly 10% of all epilepsies. It has been classified as part of »Idiopathic generalized epilepsy with variable phenotype«. The syndrome presents between the ages of 6 and 22. Clinical manifestations encompass myoclonic jerks, absence and generalized tonic-clonic seizures. Diagnose is often delayed, because characteristic clinical picture develops gradually over a few years time. Juvenile myoclonic epilepsy diagnosis is based upon clinical criteria, normal neurological examination and brain magnetic resonance imaging, and an abnormal electroencephalogram recording showing generalized discharges of multiple spike and polyspike and wave complexes with frequency higher than 3 c/sec. Recognition of myoclonic jerks is important for the diagnosis and physicians should actively seek the information on myoclonic seizures. Patients rarely complain about them. Generalized tonic-clonic seizures on awakening and juvenile absence epilepsy are high up on the list of differential diagnoses. Juvenile myoclonic epilepsy is considered to respond well to therapy and to have a good prognosis. Treatment is based on the avoidance of precipitating factors and antiepileptic drugs. Valproate is considered the first line treatment and newer antiepileptic drugs are also found effective. When a favorable response is not obtained by monotherapy, polytherapy is considered. In this review article we present the heterogeneous clinical manifestations of the syndrome, diagnostic procedures and therapeutic options. We also present the advances in neuroimaging and highlight the current trends in research.