Lyme borreliosis in children is a multi-organ infection caused by at least four genospecies of the Borrelia burgdorferi sensu lato group (B. burgdorferi sensu stricto, B. garinii, B. afzelii, and B. spielmani). Common skin manifestations of the recent stage of Lyme disease include erythema migrans and borrelia lymphocytoma. Recent extracutaneous manifestations of Lyme borreliosis are characterized by neuroborreliosis and arthritis. In children, the symptoms of the late stage of Lyme disease including acrodermatitis chronica atrophicans are rare. Prevention, early diagnosis, and therapy of Lyme borreliosis are key to preventing long-term multisystem complications.