OBJECTIVES: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children, affecting 1 in 1000.  Treatment is shifting to more costly drugs and no recent review is available that summarizes all costs for JIA.  We aimed to summarize all cost-of-illness studies for JIA. METHODS: MEDLINE and EMBASE were searched from inception to December 2013, using terms for cost-of-illness and arthritis.  Review articles were also examined.  Studies that were not published in English were excluded.  Data extracted included perspective, data sources, analysis, number of subjects, costs and year reported.  Purchasing power parities from the Organization for Economic Co-operation and Development were used to convert costs into United States dollars and the medical component of the US Consumer Price Index was used to convert costs to constant US dollars (2012).  Data are presented as cost per person per year. RESULTS: The search yielded 510 unique studies.  Nine relevant studies were identified with data from 1,340 patients with JIA.  Studies were conducted in Europe (n=5), Canada (n=2), USA (n=1) and Turkey (n=1).  Five studies surveyed patients’ families; 2 used medical records; 2 used both.  Six studies reported mean direct medical costs; range:  $3,304 to $20,613.  Six reported mean patient/parent time costs; range: $112 to $5,346.  Drug costs were $4,665 to $14,850 for studies that included newer biologic drugs (n=2) versus $353 to $1,158 for those without (n=2); direct medical costs were $5,140 to $17,633 for those with biologic drugs (n=3) versus $3,304 to $20,613 for those without (n=4).  There was inconsistency in how costs were reported. CONCLUSIONS: The economic impact of JIA is considerable.  Newer biologic drugs impact cost-of-illness estimates and must be considered when interpreting this information.  Current data largely reflects European and North American costs.  More research will assist policy developers and decision makers.