Introduction. The term epileptic encephalopathies are severe brain disorders of early age with a different manifestation, depending on the age of onset, developmental outcome, etiologies, neuropsychological deficits, electroencephalographic (EEG) patterns, seizure types, and prognosis, but all may have a significant impact on neurological development. Doose syndrome, otherwise traditionally known as myoclonic astatic epilepsy is an epileptic encephalopathy with multiple seizure types. About a third of child ren may have episodes of convulsive status epilepticus. The disease is characterized by the following criteria: genetic predisposition (high incidence of seizures and/or genetic EEG patterns in relatives); mostly normal development and no neurological defi cits before onset; primarily generalized myoclonic, astatic or myoclonic astatic seizures, short absences and mostly generalized tonic clonic seizures; no tonic seizures or tonic drop attacks during daytime, generalized EEG patterns, and often normal neuro imaging . The prognosis is variable and difficult to predict, and the seizures may remit in 54 89% of patients. Aim of the study. Review of new data about epileptic encephalopathies and clinical presentation of illustrative case of Doose syndrome Material s and methods. There are used data from literature and clinical case from our clinic. Results. In our clinical case the the diagnosis was based on the description of the seizures myoclonic and atonic seizures,mainly in the morning,and also,the patient oft en had myoclonic status epilepticus, at EEG we found focal and generalized epileptiform activity, at MRI of the brain the structures of hippocampus were different on the left compare to right ,main reason because of lost of height and width of the hippo campus structure on the left,and psychological testing severe cognitive disturbances. The treatment is adjusted permanently with raising doses of combined antiepileptic therapy, especially Valproat and Levetiracetam. Conclusions. The good news for childre n with Doose syndrome are doing better now than in the past. Outcomes have improved over the years due to early diagnosis and better treatment options.