Objective To help early diagnosis and efficacious treatment of central nervous system(CNS) aspergillosis, we reviewed 39 cases in China since 2001. Methods We collected and analyzed retrospectively the information of patients' sex and aged composition, geographical features, clinical manifestations, associated diseases, therapeutic methods and outcomes. Results CNS aspergillosis could involve immunocompromised patients and immunocompetent patients, predominantly in middle aged men. The infection of CNS often resulted from the dissemination of the Aspergillus from lungs and paranasal sinuses. There were no early specific symptoms. The chief complaint was usually the symptoms of increased intracranial pressure, with half fever(51.28%). The combination of antifungal therapy with neurosurgery seems to be the most efficacious approach, and voriconazole was the best one. This disease had a poor prognosis, with high mortality. Conclusions With atypical clinical symptoms, nonspecific laboratory or imaging findings, and difficult diagnosis, CNS aspergillosis was still a disease with high mortality.