A 65-year-old woman showed progressive asymmetric sensory ataxic neuropathy (SAN) in upper limbs over 12 years, beginning with the onset of primary biliary cirrhosis (PBC). Nerve conduction study showed that sensory action potential was not evoked in upper limbs. Right superficial radial nerve biopsy showed the density of myelinated fibers was severely decreased with large-fiber predominance. Since Sjögren's syndrome (SjS) and paraneoplastic syndrome were denied, this type of neuropathy seems accompanied with asymptomatic PBC. Corticosteroid administration and intravenous immunoglobulin therapy failed to obtain any beneficial effect. The pathological mechanism is considered ganglionitis, which resembles that found in association with SjS. We should also consider PBC as the cause of SAN.