Hydatidosis is a zoonotic disease caused by Echinococcus parasite that frequently involves liver and lungs. Primary intracranial hydatidosis is a rare condition which can be life threatening if ruptured. Here we report an unusual case of primary intracranial hydatid cyst without any other organ involvement, diagnosed in a 12-year-old boy in Emam Reza hospital, Birjand, Iran in November 2016, in order to focus on the importance of proper diagnosis and management, especially in endemic areas.