Intracranial hydatidosis is a rare parasitic disease secondary to human intracranial development of the larval cysts of the tapeworm Echinococcus granulosus. The cyst, which is usually encephalic, spherical, solitary, subcortical, and supratentorial, is most often seen in children or young men. The most common presenting symptoms are those of intracranial hypertension, neurologic deficits, seizures, and mental changes. The cyst is well limited, with a density like CSF on CT scans and signal intensity like CSF on MRI. Surgical removal of the intact cyst is the cornerstone of treatment. The cyst is frequently delivered via a hydrostatic expulsion (Dowling’s technique). Every effort should be made to deliver the intact cyst without rupture. Anthelmintic treatment may be considered to control the disease locally, avoid systemic spread, and prevent recurrence. Recovery from neurologic deficits occurs in most patients, and the mortality rate is less than 10%. Recurrence is common, nearly always owing to spillage of cystic contents at the time of removal of the original cyst or in patients with multiple cysts.