Hydatid disease is caused by Echinococcus granulosus. The liver is the first organ of entry, while the central nervous system is affected in about 2% of cases. Primary brain hydatid cyst is extremely rare. The cysts are generally located in cerebral lobes. Here, we reported a 68-year-old Syrian immigrant woman with hydatid cyst in the left cerebellopontine angle. The patient presented gait disturbance, asymmetrical palatal arcs, negative gag reflex, and disturbed cerebellar tests on the left side. The patient was operated via suboccipital craniotomy. The cyst ruptured during manipulation, but it was totally removed.
Keywords: Cerebellopontine angle; hydatid cyst.