A sural nerve biopsy was performed in a case of adrenomyeloneuropathy which was confirmed by biochemical investigations. Morphometric study of the nerve revealed a loss of large myelinated fibers with signs of demyelination, and normal unmyelinated fibers. Electron microscopic study showed typical lipid inclusions in some histiocytes and Schwann cells. The interest of nerve biopsy in histopathological diagnosis of adrenomyeloneuropathy is emphasized.