Cystic echinococcosis(CE) is a complex, chronic and neglected disease with a worldwide distribution. The liver is the most frequent location of parasitic cysts. In humans, its clinical spectrum ranges from asymptom-atic infection to severe, potentially fatal disease. Four approaches exist in the clinical management of CE: surgery, percutaneous techniques and drug treatment for active cysts, and the “watch and wait” approach for inactive cysts. Allocation of patients to these treat-ments 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. This paper reviews recent advances in classification and diagnosisand the currently available evidence for clinical deci-sion-making in cystic echinococcosis of the liver. The liver is the most frequent location of parasitic cysts. In humans, its clinical spectrum ranges from asymptom-atic infection to severe, potentially fatal disease. Four approaches exist in the clinical management of CE: surgery, percutaneous techniques and drug treatment for active cysts, and the “watch and wait” approach for inactive cysts. Allocation of patients to be treat based on cyst stage, size and However, clinical decision algorithms, efficacy, relapse rates, and costs have never been well evaluated. This paper reviews recent advances in classification and diagnosis and the currently available evidence for clinical deci- sion-making in cystic echinococcosis of the liver.