Cystic echinococcosis, or cystic hydatidosis, is a complex, chronic disease with a cosmopolitan distribution. In humans, its clinical spectrum ranges from asymptomatic infection to severe, rarely even fatal disease. Four approaches in clinical management exist: surgery, percutaneous techniques and drug treatment for active cysts, and the so-called watch and wait approach for inactive cysts. Allocation of patients to these treatments should be based on cyst stage, size and location, available clinical expertise and comorbidities. However, clinical decision algorithms, efficacy, relapse rates, and costs have never been properly evaluated.We review the currently available evidence for clinical decision-making and discuss ways to improve standards of care of one of the most neglected infectious diseases. Data are mostly derived from case series and small clinical trials, and treatment guidelines remain at the level of expert opinion. No single high-quality comparative clinical trial of the four treatment options is available to resolve important questions such as stage-specific allocation of treatments, adverse events and long-term relapse rates. Recent work is beginning to acknowledge this problem. Currently, four treatment modalities are available for cystic echinococcosis. The level of evidence on which clinicians have to rely is low. For the time being patients should thus be treated in referral centres. Proper comparative clinical trials are urgently needed.