OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics. RESULTS: In total, 64 subjects with jSSc were enrolled a median of 3.6 years after disease onset, which occurred at a median age of 10.3 years old. The most common organ manifestations were dermatologic and vascular, followed by musculoskeletal, gastrointestinal, and pulmonary, with 38% of patients having more than four organ systems affected. Patients with jSSc had significantly more disability at enrollment than CARRA registry patients with juvenile idiopathic arthritis, dermatomyositis, or systemic lupus. While physician reported measures correlated most closely with arthritis, skin, and pulmonary manifestations, poor patient-reported measures were associated with gastrointestinal involvement. Over 50 person-years (median duration 1.4 years), there was stability of most organ manifestations with no mortalities or development of new solid organ involvement after enrollment. CONCLUSION: In the first multicenter prospective cohort of juvenile SSc patients in North America, disease burden was large: multi-organ manifestations were common, and functional disability was greater than other childhood rheumatic diseases. Gastrointestinal involvement had the greatest impact on quality of life. This article is protected by copyright. All rights reserved.