Syndrome Aicardi-Goutières is a rare inherited disease characterized by subacute onset encephalopathy, calcifications located in basal ganglia, leukodystrophy, cerebrospinal fluid lymphocytosis and elevated interferon levels. Behind the complex etiology, seven gene mutations, inflammatory and autoimmune processes have been discovered so far. The latter, categorized this syndrome in the group of interferonopathies, today increasingly present pathogenesis in a number of autoimmune diseases. Human interferons are classified into three types depending on the receptor to which they bind. The type I interferon family is of particular importance here, interferon alfa respectively. Symptoms commonly begin in infancy after the normal onset of development, and clinical presentation can be divided into five groups. Although specifics have been reported for each mutation, in most patients after several months of active disease, further progression is halted, leaving severe physical and intellectual disability in patients. In addition to expression of interferons and genes stimulated by them, neuroradiological findings are also important in diagnosis, additionally contributing to the differentiation from primarily prenatally acquired infections. Therapeutic options are for now mostly symptomatic, including the treatment of concomitant diseases. A more detailed understanding of intracellular mechanism leaves a great potential for the development of new drugs, the application of which at an early stage could reduce inflammation and its effect on tissues.