Rare neurometabolic and neurodegenerative diseases of the brain include a wide diagnostic scale of diseases which generally have a serious, often sinister course. In this chapter we focus on metabolic diseases marked by depositing metal molecules, lysosomal storage diseases, Huntingto's disease, rare dystonic syndromes, motoric neuron disorders, spinocerebellar ataxias in both childhood and adulthood and some rare neuro-developmental disorders.