The malformation was first described in 1883 by dr. Cleland, in an article entitled Contribution to the study of spina bifida, encephalocele, and anencephalus ("Contribution to the study of spina bifida, encephalocele, and anencephaly") in the Journal of Anatomy and Physiology. Eight years later, the neurologist austrian Hans Chiari wrote an article in the Deutsche Medizinische Wochenschriff entitled "About the alterations in cerebellar resulting from hydrocephalus brain", in which he acknowledged the work of Cleland and added his own system of classification of this disorder into types I, II and III In 1896, Chiari described the disease as follows: "An elongation in the form of a wedge of the cerebellar tonsils and the medial part of the lower lobes of the cerebellum, that run the length of the cord within the cervical canal".
In 1907, Schwalbe and Gredig, two students from a team from the University of Heidelberg led by dr. Julius Arnold described a total of four other cases, prepending the name of his master to the Chiari in the designation of the malformation.