Systemic onset juvenile idiopathic arthritis (SoJIA) remains difficult to treat. In addition to conventional antirheumatic therapy with non-steroidal antirheumatic drugs (NSARDs), steroids or disease-modifying antirheumatic drugs (DMARDs), biologicals offer a new therapeutic approach for this disease in that they are able to target pathogenically relevant cytokines and effector cells. Some biologicals are already approved for use in children with rheumatic disease.In order to assess the currently available data on the use of biologicals in SoJIA, we performed a Medline search for the period 2005 to March 2010, including the MeSH terms "SoJIA", "systemic juvenile idiopathic arthritis" and"biologicals", as well as an NIH study registry search. At Present there are scant and unconvincing data on the use of Etanercept or Adalimumab for the treatment of SoJIA. No results are published on the use of Infliximab or other new TNF-alpha inhibitors. The inhibition of IL-1 or IL-6 shows promising results. Data on the efficacy of Abatacept is limited due to very low numbers of SoJIA patients in the studies.Further studies on the use of biologicals in SoJIA while taking individual factors into consideration are required. The long-term safety of all biologicals should be investigated in prospective registers.