Innovative developments in the pharmacotherapy of juvenile idiopathic arthritis (JIA) not only allowed the avoidance of long-term damage and disability but also the rapid induction of remission with shortening of the period of active disease. In this article the treatment options are discussed on the basis of clinical trials and long-term documentation of the treatment experience. While in oligoarticular JIA (oligo-JIA) intra-articular corticosteroids and non-steroidal anti-inflammatory drugs (NSAID) are initially used, patients with rheumatoid factor positive and negative polyarthritis, extended oligoarthritis (extended oligoarticular juvenile idiopathic arthritis, eoJIA) and polyarticular psoriatic arthritis (PsA) receive early treatment with disease-modifying drugs. Unless contraindicated, methotrexate (MTX) is the drug of first choice, while sulfasalazine has proven to be effective in enthesitis-related arthritis (ERA). Failure to achieve the therapeutic goal after 3-6 months prompts an escalation of pharmaceutical treatment including the use of biologics. For children above 2 years of age, the tumor necrosis factor (TNF) inhibitors etanercept and adalimumab, and the interleukin 6 (IL-6) receptor antibody tocilizumab are available. In cases of inadequate response, a change in therapy is also contemplated in short 3-6 month intervals with the aim of remission induction. In the treatment of systemic juvenile idiopathic arthritis (Still's disease, sJIA) a distinction is made between a more systemic and articular disease patterns. While the latter is treated in the same way as polyarthritis, systemically active courses in patients with limited response to NSAIDs and corticosteroids receive an early cytokine blockade with tocilizumab or the IL-1 inhibitors anakinra or canakinumab. The use of anakinra for steroid-free first-line treatment of systemic JIA is a promising, pathophysiologically targeted therapy approach but currently still to be classified as experimental. © 2014 Springer-Verlag.