The term athetosis has progressively disappeared from the anglo-saxon literature which considers that athetosis is part of the spectrum of dystonia. These two clinical entities can be distinguished, however. Athetosis can be identified, searching for subtle semiological traits, in particular at the level of the hand. The earlier appearance of athetosis may be result from its onset during the early phases of development of the central nervous system. Despite its rarity, the clinical diagnosis of athetosis is important to consider from a prognostic point of view. Indeed, it results from brain lesions, and is therefore not a hereditary disorder as it may be the case for dystonia, and its evolution is relatively stable. The efficacy of treatments used in patients with dystonia, in particular high frequency pallidal stimulation, remains to be assessed in patients with athetosis. The concept of athetosis is still helpful in clinical practice.