The clinical phenomena of "acroosteolysis" means progredient destruction starting at metatarsal bony structures on primarily neurodystrophic mechanisms. A long-term case report illustrates clinical features and actual possibilities and limits in treatment. The current conceptions since the classic descriptions are resumed by large overview of literature. Open questions in current genetic and structural hypothesis show the difficulties of any nosologic classification. Common features with other neurodegenerative syndromes are more and more evident.