Complex regional pain syndrome (CRPS) is a pain syndrome of the extremities that can result in severe disability. CRPS is diagnosed using diagnostic Budapest criteria based on signs and symptoms, whereby sensory, autonomic, vasomotor, motor and trophic disturbances are assessed. Many pathophysiological mechanisms are proposed in the development and disease course of CRPS, starting with exaggerated inflammation and resulting in vascular deregulation, central sensitisation and cortical reorganisation. Treatment is based primarily on reducing inflammation by using medicinal anti-inflammatory therapy and increasing motor function by physiotherapy. Furthermore, pain reduction, normalisation of vasomotor and motor function, and psychological interventions might be needed. Future research should focus on the efficacy of anti-inflammatory therapy, effective rehabilitation programmes, modulating neuropathic pain and cortical reorganisation.