Background: Hydatid disease is a common infection caused by Echinococcus granulosus. Cerebral involvement is very rare and only occurs in 2% of all hydatidosis in endemic areas. We report a case of primary multiple cerebral hydatid cysts in a 23 years old patient. Case: A 23 years old female was admitted in our emergency ward with primary generalized tonic colonic seizure, left body and face hemiparesis and history of headache in last two months. Contrast enhanced CT and MRI showed a big well-defined, rounded, cystic lesion in the motor strip of right temporo-parietal region with mass effect causing midline shift which was diagnostic for brain hydatid cyst and several smaller cysts. There was no evidence of other organs involvement. The patient underwent the brain surgery. A big hydatid cyst (10cm in diameter) and some others adhering to brain parenchyma in different sizes were evacuated completely and without rupture using Dowling method. Patient received albendazole 400mg/two times a day for 2 months too. There was no cyst in patient’s MRI study in two years follow up.

Conclusion: Though low incidence, primary multiple cerebral hydatid cysts should be considered in endemic area using brain CT and MRI. Surgery is standard treatment of the disease.