Objective: To analyse the epidemiological characteristics, clinical symptoms, radiological aspects, treatments, and outcomes of primary central nervous system (CNS) hydatidosis and compare our results with those observed for secondary intracranial hydatidosis. Patients and Methods: We retrospectively reviewed 21 cases of primary CNS hydatid cysts operated on at the First Affiliated Hospital of Xinjiang Medical University between 1996 and 2010. Results: Of the 21 primary cases, the vast majority were intracranial hydatidosis patients (20 cases, 95.24%). Only one patient had spinal hydatidosis. Unlike previously published reports, we found that intracranial hydatid cysts were more common in adults (80.96%) than in children (19.04%), with a slight male predominance (M/F 5 1.1). All symptoms, including vomiting, nausea, and focal neurological signs, resulted from the increased intracranial pressure, which was closely associated with the cyst location. For the spinal hydatidosis patient, the primary symptom of back pain was indicative of spinal cord compression syndrome. All cysts in the 21 primary cases were pathologically similar. The recurrence percentage was 28% over 12 years. Two patients with multiple intracranial hydatid cysts died due to foramen magnum herniation. Conclusion: Despite imaging and therapeutic advances, CNS hydatidosis remains difficult to treat, and severe complications and the high incidence of recurrence result in unsatisfactory outcomes.