We report on a 61-year-old patient with sclerodermiform skin lesions of the extremities and polyneuropathy. Borrelia (B.) burgdorferi DNA was detected in lesional skin by the polymerase chain reaction. Serological testing revealed IgG antibodies to B. burgdorferi. Histology revealed an inflammatory stage of a sclerotic reaction in the lesional skin. The admixture of plasma cells and the perineural distribution of the cellular infiltrate was suggestive for a borrelia infection. Immunohistochemical staining for the B. burgdorferi flagellin (41 kDa) revealed a positive staining reaction in the epidermis of lesional skin. The improvement of both the dermatological and the neurological symptoms upon antibiotic treatment with ceftriaxone was taken as further support for the diagnosis of a. B. burgdorferi infection.