Acrodermatitis chronica atrophicans is a rare late manifestation of tick-borne Borrelia burgdorferi infection, manifesting as inflammatory and atrophic lesions on acral skin. We describe the case of a 73-year-old woman with skin changes progressed to marked atrophy on her left hand and an edematous inflammatory involvement of the face. The diagnosis of acrodermatitis chronica atrophicans was made on the basis of clinical appearance, serological and histopathological findings, and the lesional detection of B. burgdorferi-specific gene segments by polymerase chain reaction. This unusual case illustrates that acrodermatitis chronica atrophicans affects not only the extremities but also the face. The clinical and histological finding of the lesions occurring on acral skin showed a prominent atrophic appearance, while the ones occurring on the face showed a prominent inflammatory appearance.