Heretofore unreported findings in skeletal muscle in a 4th case of pure postganglionic dysautonomia are described. They consist of variation in fiber size, Z line and myofilamentous disorganization, intracytoplasmic inclusions and honeycomb formations. The most striking alteration, however, was excess lipid accumulation without concomitant mitochondrial alterations. Sural nerve histology and cytology were normal. The mechanism of lipid accumulation in skeletal muscle of patients with dysautonomia remains unexplained.