Microangiopathies are progressive genetically determined diseases of the cerebral small blood vessels. Usually, they are generalised vascular diseases, only with the dominance of symptoms of the central nervous system damage. Characteristic symptoms are recurrent ischaemic or haemorrhagic strokes and a variety of neurological symptoms such as migraines, epilepsy, early cognitive disorders, mental disorders as well as specific changes in neuroimaging and neuropathological findings in young patients. In magnetic resonance imaging of the brain, diffused or focal hyperintensities in the cerebral white matter are visible, and in biopsy specimens characteristic histopathological changes in vessels are observed. The most well-known and common microangiopathy is CADASIL, but the literature devoted to cerebrovascular disorders is rapidly updated with new diseases and syndromes. Thanks to the better accessibility of neuroimaging methods and histopathological assessment of biopsy materials, microangiopathies are increasingly recognised. Early diagnosis of microangiopathies is of great practical importance, as not only does it prevent unnecessary additional tests, but also requires modification of routine treatment.