The skin is the largest organ comprised of three main layers: epidermis, dermis and subcutaneous tissue. Characteristic microscopic and ultrastructural alterations are present in certain skin structures, such as nerve fibers, blood vessels and sweat glands, not only in vasculitis, but in several other metabolic neurologic diseases. In recent years, advances in immunohistochemistry and electron microscopy have made these dermatopathological changes identifiable and clear-cut. It was then recognized that such pathological feature could be present in many tissues in addition to those nerve systems clinically affected. Skin biopsy has become a recognized diagnostic procedure for peripheral neuropathy and central nervous system diseases. This article reviews the dermatopathology in neurological disorders under light and electron microscope to promote application of skin biopsy in clinical diagnosis for neurologist.