During the past 10 years, descriptions of large registries of children with juvenile systemic sclerosis (jSSc) have improved our knowledge of this disease. jSSc differs from the adult disease in presentation as well as disease course. Two courses of disease have been described: a rapidly progressive and fatal illness with cardiac involvement; and, more commonly, a chronic course with less overall mortality. Subclinical disease, especially cardiac and pulmonary disease, should be specifically sought and appropriately monitored, particularly in the first years of disease. Use of screening pulmonary function tests and high-resolution CT increases the chance of detecting interstitial lung disease. Close attention needs to be paid to cardiac health, with improved understanding of the specific causes of death to aid in the development of preventive measures. Therapies that best balance risks and benefits are likely to differ between children and adults. Given the relative rarity of jSSc, pediatric trials will require multinational collaborative efforts.