A 66-year-old woman presented with a 2-year history of acrodermatitis chonica atrophicans of her left hand and associated fibrous nodules. In addition to skin changes she experienced profound fatigue, and pains and swelling of the left elbow and dorsum of the left hand. On clinical examination, typical livid-red discoloration of the skin distally from the left elbow was evident with atrophy on the dorsum of the hand. Nodules with diameters from 0.5 to 2 cm were present around the olecranon and along the ulnar region. The indirect immunofluorescent assay (IFA) without absorption in serum revealed negative borrelial IgM and positive IgG (1:512) antibody titres. Histological findings on tissue specimens were compatible with acrodermatitis chronica atrophicans and fibrous nodule tissue, respectively. Cultures in MKP medium of biopsy specimens from the involved skin and from one of the nodules were positive. Both isolates were identified as B. afzelii with concordant protein and plasmid profiles. The patient received a 3-week course of ceftriaxone (2 g daily, i.v.). Her condition progressively improved: pains and swelling vanished by the end of treatment, fibrous nodules diminished and skin lesions gradually began to fade. According to a MEDLINE literature search this is the first report of the isolation of B. burgdorferi sensu lato from a fibrous nodule in a patient with acrodermatitis chronica atrophicans.