Human hydatidosis (cystic echinococcosis) is a parasitic infection still endemic in several countries of the world, particularly in rural areas posing a potential public health problem. The most frequent and classic localizations of human echinococcosis are the liver (75%) and the lungs (15%), but any other sites/organs may be involved by hydatid cyst. Central nervous system (CNS) involvement during this parasitosis (neurohydatidosis) is considered among the so-called “unusual", “ectopic" or “aberrant" locations.  CNS hydatidosis is divided into two major nosological frameworks: brain and spinal involvement. Brain hydatidosis is exceptional with a prevalence estimated at less than 2% of all human hydatidosis, and the majority of cases are reported as sporadic observations.
This localization represents a real diagnostic challenge for clinicians, even in endemic areas. It is characterized by its severity, these heavy functional repercussions and can sometimes be fatal.
The purpose of this review is to familiarize hospital practitioners with this unusual location of hydatid cyst, to avoid its delayed diagnosis and improve its prognosis.