Echinococcosis is an infectious disease that can remain dormant for years. The most common sites of infection are liver and lungs. Primary cerebral disease is very rare. Here we report on an unusual case of echinococcosis, where the only identifiable lesion was a slowly growing intracranial hydatid cyst. No lesions on the liver identified. The patient is a United States immigrant from rural South India with a chief complaint of progressive weakness and aphasia. Further analysis revealed an interesting association between the clinical, anatomical and hematological findings over time. We conclude that hydatid cyst disease should be considered in patients from endemic areas with long-standing neurological symptoms.