A case of idiopathic orthostatic hypotension (IOH) is presented with clinical and autopsy data. Electron microscopic examination identifies previously described eosinophil bodies in the sympathetic ganglia as axonal swellings. The case presented is correlated with data from literature. Neuropathologic findings include degenerative changes in several systems, with intermediolateral column cell loss the only change common to all the reported cases. The relationship of IOH to other degenerative diseases of the central nervous system is discussed.