Hydatidosis is both a medical and an economic problem in Turkey. The aim of this study was to analyze central nervous system (CNS) involvement in this disease, the related problems the disease causes, and its diagnostic and therapeutic aspects. The authors conducted an extensive literature survey of the subject, in which papers published by Turkish authors in international and domestic journals were carefully analyzed. In addition, the authors conducted a cooperative study in which data were gathered from 47 neurosurgery departments across the country. The purpose was to determine the current status of the disease in Turkey; thus, each unit was questioned about their experience over the past 5 years. Contrary to common belief, the incidence of hydatidosis has not decreased significantly in Turkey. However, computerized tomography and magnetic resonance imaging have tremendously increased diagnostic specificity. Incidences of morbidity and mortality have improved over time, according to the results of the cooperative study, although these changes are not statistically significant. This may be attributed to experience that has been gained and to more frequent use of chemotherapy, as reflected by the cooperative study data. The two statistically significant findings of that study were expanded use of chemotherapy in the management of hydatidosis, and a higher rate of extraneural involvement in the disease. The cooperative study revealed that chemotherapy was being used more often and that there was a wider range of indications for this treatment than previously reported. The higher rate of extraneural involvement was predictable because lesions in the CNS are typically secondary in this disease. With regard to the studies' findings on cases of spinal hydatid cysts, the authors found that administration of chemotherapeutic drugs was the only statistically significant parameter (t = 3.78, p < 0.05), with the rate of chemotherapy higher in the cooperative study. Morbidity, mortality, and recurrence rates of hydatidosis uncovered by the cooperative study and the literature survey were not statistically significant.