© The Author(s) 2018. Juvenile localised scleroderma is believed an orphan autoimmune disease, which occurs 10 times more often than systemic sclerosis in childhood and is believed to have a prevalence of 1 per 100,000 children. To gain data regarding the prevalence of juvenile localised scleroderma, we assessed the administrative claims data in the United States using the International Classification of Diseases, Ninth Revision diagnosis codes. We found an estimated prevalence in each year ranging from 3.2 to 3.6 per 10,000 children. This estimate is significantly higher as found in previous studies.