We report a case of acrodermatitis chronica atrophicans in an 11-year-old girl living in an area endemic for Lyme borreliosis. The diagnosis was first made on the basis of clinical, histopathological and serological findings. Moreover, Borrelia burgdorferi-specific DNA was amplified from lesional skin by polymerase chain reaction. Intravenous treatment with ceftriaxone (2 g once daily) for 2 weeks was initiated. The skin changes clearly responded to the therapy, and Borrelia burgdorferi-specific gene segments were no longer detectable by polymerase chain reaction. This is the first report of molecular-proven acrodermatitis chronica atrophicans in childhood. The occurrence of this late skin manifestation of Lyme borreliosis in children is reviewed.