Autoimmune epilepsy in children is a new clinical entity which is now considered separate from autoimmune encephalitis. Characteristics of autoimmune epilepsy are seizures resistant to treatment with antiepileptic drugs, autoantibodies targeting intracellular antigens and autoantibodies targeting plasma membrane proteins (cell-surface autoantibodies), positive cerebrospinal fluid findings suggestive of inflammation, imaging evidence of central nervous system inflammation and positive response to corticosteroid and immunomodulatory/immunosupressive therapy. Cell-surface antibodies have demonstrated pathogenic potential and this is considered a dominant pathogenic mechanism. The detection of these autoantibodies can lead toward the possible cause of epilepsy such as cancer or infective agent. It is important to emphasize that the detection of autoantibodies is not necessary to establish the diagnosis of autoimmune epilepsy. Triggers of autoimmune process in most cases remain unidentified. Some patients present primarily with seizures resistant to treatment with antiepileptic drugs, without other signs of autoimmune disease of central nervous system, but in most cases seizures are associated with symptoms of autoimmune encephalitis. Autoimmune epilepsy is suspected if a child already has a known systemic autoimmune disease. Not many research related to children has been conducted, but there is a consensus and certain diagnostic and therapeutic guidelines are given how to act in cases with suspected autoimmune epilepsy. It is important to discover autoimmune origin of seizures because the disease can be treated with corticosteroids and immunoglobulins and in most cases there is a good response to the treatment (at least on short term). Early recognition of autoimmune epilepsy is important because an early start with immunomodulatory therapy has better outcomes and no severe neurological consequences. The response to immunotherapy can support the diagnosis of autoimmune epilepsy and determine the prognosis.