▼ Complex regional pain syndrome (CRPS) typically develops following extremity injuries and is characterised by distally generalised sensory, motor, trophic, vaso-and sudomotor dysfunctions, as well as joint affl iction. CRPS cases without nerve lesions are defi ned as type I -those involving nerve damage as type II. The diagnosis is primarily based on the clinical presentation using the so called 'Budapest criteria'. Important for the diagnosis is the exclusion of other potential conditions which could account for the patient-reported symptoms and demonstrable clinical signs. Triple-phase bone scans are helpful for the early and diff erential diagnosis due to their high specifi city (98 %), although their sensitivity decreases as the disease progresses. In the acute stage of the disease, pro-infl ammatory factors seem to play an important role, allowing for a potential therapeutic approach. As the disease progresses, however, they are unrelated to the clinical severity. Chronic stages are primarily determined by central nervous system changes including a reduced cortical representation of the aff ected limb, changes in body scheme, neglectlike symptoms, as well as dystonic deformities. The most common sensory abnormality is pressure hyperalgesia over muscles, most likely a result of peripheral sensitisation. In addition to the clinically detectable focal autonomic disorders, more generalised autonomic dysfunction is also found. The pathogenesis of dystonic symptoms, however, remains poorly understood, making therapeutic treatment more diffi cult. Treatment of CRPS should be multimodal and take place in a multi-disciplinary setting, adapted to the level of pain. Invasive interventions should be limited to strict indications and specialised centres.