Among 78 cases of alveolar echinococcosis reported in Lorraine, France, 5 had neurologic complications which in 3 cases revealed the disease. Results of parasitic tests are discussed and emphasis is placed on differences between this disease and hydatidosis. Encephalic localizations (3 cases) were multiple making neurosurgery impossible. The outcome was fatal in 2 cases: 1 month after the initial neurologic signs in the absence of treatment (case 1) and 4 months after treatment with flubendazole (case 2). This drug was however effective in the 3rd case (hepatic, pulmonary and cerebral form) with follow-up now at 4 years. Epidemiologic, histopathologic, clinical, diagnostic characteristics and course of these encephalic localizations are reviewed. Spinal localizations (2 cases) presented with a picture of spinal cord compression. After laminectomy and flubendazole, the course was marked by relapse with death in one case and a satisfactory neurologic course with a 4 year follow-up in the other one.