• Sensory symptoms can develop in the course of primary biliary cirrhosis. They have been attributed to xanthomatous infiltrates of the nerves. We report a case of a young woman suffering from pure sensory neuropathy starting at a time when evidence of liver disease was minimal. Histologic and morphometric studies of sural nerve biopsy specimens obtained from calf and ankle showed a dying-back type of axonal degeneration predominantly affecting large myelinated fibers. No lipid-containing cells were seen, which raises the possibility that other mechanisms related to primary biliary cirrhosis are involved in the pathogenesis of the neuropathy.