Brain hydatid cyst constitutes one of the leading causes of cranial mass in the endemic region, especially among the children. Symptoms of the disease are associated with rising of intracranial pressure and focal neurological deficit. Surgical excision of the cyst is essential to treatment. Here we present a 19 yr-old male livings in Ardabil, northwest of Iran, referred to Neurosurgery Clinic in Khanevadeh University Hospital, Tehran, Iran with a large brain hydatid cyst and atypical symptoms. He was treated by surgical excision of the cyst without any complication. There were not any clinical or radiological signs of recurrence after one-year follow-up.