The role of praziquantel in hydatid disease has not been well defined. This review evaluates the evidence on the use of praziquantel in treatment of cystic hydatid disease from in vitro and in vivo animal studies, human clinical studies and case reports. Praziquantel may prevent the vesicular evolution of protoscoleces and inhibit the formation of secondary cysts. It may also contribute to the loss of viability of small cysts before cyst differentiation and development of the fibrous adventitial layer. There is some evidence to support a role for the use of praziquantel in combination with albendazole in pre- and post-intervention chemotherapy for hydatid disease. Combined therapy may reduce the risk of disease recurrence and intraperitoneal seeding of infection that develops via cyst rupture and spillage occurring spontaneously or during surgery or percutaneous procedures. At present, there is insufficient published evidence to support a clear recommendation for the use of praziquantel in prolonged chemotherapy for established hydatid disease for which surgery is not indicated or in severe disseminated disease and further work is necessary. Randomised controlled studies to determine the efficacy and optimum duration of praziquantel treatment in combination with albendazole are required so that treatment recommendations for its use can finally be clarified.