Introduction: Hydatid disease is caused most common by Echinococcus granulosus and Echinococcus multilocularis. The former is the most common and is endemic in areas such as Australia, New Zealand, the Mediterranean, the Middle East, and South America. Typical presentations of hydatid disease have been frequently described in the literature; however, uncommon presentations have not been thoroughly documented.

Case Presentation: Here, we report three rare but well-documented cases of central nervous system hydatid cysts that occurred in patients in Iran.

Conclusions: We also provide a brief review of the literature examining similar occurrences. This article intends to provide thorough information about the disease for readers.