In this chapter, drug treatment in the few treatable causes of degenerative ataxias will first be presented including vitamin deficiency, metabolic disorders, and autoimmune disorders. Next, older and more recent drug trials will be discussed which have been performed in Friedreich’s ataxia and may lead to effective drug treatments in the future. Finally, it will be shown that, as yet, symptomatic treatments of most degenerative ataxias are lacking, that is, there is still no “anti-ataxic drug.” The only exceptions are aminopyridines and acetazolamide which can be beneficial in the treatment of downbeat nystagmus and episodic ataxias.