Abstract
It is shown in the example of a 36 year old female patient with progressive but mainly carpotarsal osteolysis to analyse the radiological and clinical delimitation to other osteolysis-syndromes. Over 35 years during infancy and adolescence the clinical picture was incorrectly related to the "Freeman-Sheldon-Syndrome" and was twice the cause for fruitless attempts to classify. Although no standardized classification or rather grouping of the osteolysis exist, now with means of systematically radiological diagnostic and by long clinical follow-up of over 35 years a definite enrollment of the case represented, proved to be an extreme rare form of idiopathic multicentric osteolysis with nephropathy. It can be listed now as the 13th case in the literature.
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