Systemic Lupus Erythematosus (SLE) is an autoimmune, multisystemic, chronic disease that is difficult to diagnose. Few studies describe its features in the South American pediatric population. Objective: to describe clinical and laboratory features, course, and treatment of childhood-onset SLE patients and their transition into adulthood. Patients and Method: Retrospective study of patients diagnosed with SLE in a Children's Rheumatology Unit of a hospital in Santiago de Chile between 2001 and 2017. Epidemiological, clinical, laboratory, treatment received, evolution, complications and hospitalizations data were registered. It was considered severe SLE the cases with renal or central nervous system involvement. Results: 31 patients were studied, all with the disease longer than 6 months. The female/male ratio was 5.2/1. The median age of presentation was 12.5 years. In 94% of cases, the diagnostic delay was less than 6 months. The most frequent clinical characteristics were arthritis (87%), skin lesions (58%), and renal involvement (58%). The most frequent laboratory findings were positive antinuclear antibodies (100%), positive anti-dsDNA antibodies (74%), and hypocomplementemia (71%). Corticosteroids, hydroxychloroquine, and mycophenolate were the most commonly used drugs. There was no mortality in this group. 97% of patients had "satisfactory check-ups" during pediatric care and 59% in the adult one. The transition was scheduled in most cases. Conclusions: The results of this study were similar to other publications and is one of the few studies describing SLE in the Chilean pediatric population. In addition, it describes the transition into adulthood.