Parry-Romberg syndrome, which is also known as progressive facial hemiatrophy, is a variant of localized scleroderma. This is a rare condition (OMIM # 141300), characterized by a progressive but self-limited atrophy of the skin and subcutaneous tissue on one side of the face. We present the case of a 9 year old boy with a typical presentation of hemifacial atrophy, with Borrelia burgdorferi infection without any changes of the nervous system.