Cystic echinococcosis (CE) is a neglected zoonosis caused by the larval stage of the cestode Echinococcus granulosus. It represents one of the major health priorities in developing countries and its global prevalence is about three million. E. granulous modulates anti-parasite immunity and persists in infected humans despite the occurrence of detectable parasite-specific humoral and cellular responses. In particular, the up-regulation of the Th2-type response and of anti-inflammatory cytokines production is involved in the chronicity of the infection and in the inhibition of host protective mechanisms. In this chapter, we underline findings about the human immune response during the development of CE and new insights regarding to the ability of E. granulosus to directly modulate and even to exploit the host’s immune system. New knowledge into the host immune response to the parasite will increase our understanding of this parasitic infection and will give the opportunity to design new preventive or therapeutic strategies.