Clinical and/or neurophysiological signs of peripheral neuropathy were found in 64% of 63 consecutive untreated patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). The neuropathy frequency was significantly higher in the patients than in 30 age- and sex-matched control persons of whom 27% had neuropathy findings. The most common neuropathy in ACA was a symmetric distal sensory polyneuropathy. In a subgroup of patients with localized or asymmetric neuropathy, the changes were found more often in extremities with than without visible ACA lesions. Neuropathy symptoms, most often pain and/or paresthesia, were present in 64% of the patients, compared to in 13% of the control persons. Thus, both symptoms and signs of neuropathy were significantly more frequent in patients with untreated ACA than in control subjects.