Spinocerebellar ataxias are a group of hereditary neurodegenerative disorders caused by a change in a number of repetitive DNA sequences in a particular gene, i.e. dynamic mutation. These changes cause progressive degeneration of Purkinje cells in cerebellum and neurons of brainstem, which leads to the emergence of typical symptoms: ataxia of gait, posture and limbs, dysarthria, pyramidal and extrapyramidal disorders, amyotrophy, cognitive impairment etc. Currently, treatment of this group of diseases is purely symptomatic. However, research into these disorders resulted in the discovery of molecular pathways and potential therapeutic targets, among others: suppression of neuronal intranuclear inclusions formation, activation of ubiquitin/proteasome pathway, inhibition of caspases and preventing excytotoxicity and oxidative stress. Clinical use of this knowledge may provide the means to developing effective therapeutic strategy of these currently incurable diseases.