BACKGROUND: Central nervous system (CNS) involvement is a rare but serious complication of fungal infection with increasing incidence, especially in immunocompromised patients. Candida spp., Cryptococcus spp. Aspergillus spp. and Mucorales spp. are the most common pathogens. Despite continuous advancement, diagnosing remains challenging. This can lead to significant delays in diagnosis and therapy, thereby negatively affecting outcomes. To date, data on clinical symptoms, diagnostics and treatment of CNS fungal infections remain scarce. METHODS: We retrospectively analyzed data from patients ≥ 18 years old with CNS fungal infection treated between 01/01/2007 and 31/12/2023 in the Departments of Neurology, Neurosurgery, and Oncology at the University Hospital of LMU, Munich. Data included biometrical data, clinical symptoms, laboratory and microbiological results, brain imaging, treatment, and clinical course. RESULTS: 58 patients with CNS fungal infections were identified. Our key findings were: (1) the most common fungus was Aspergillus spp, followed by Cryptococcus spp, Mucoracae spp and Candida spp
(2) clinical symptoms and immunosuppressive factors varied between pathogens
(3) candidiasis and cryptococcosis mostly manifested as meningitis, while mucormycosis and aspergillosis presented as mass lesions
(4) time to diagnosis was longer for Candidiasis patients than for other fungal infections
(5) antifungal regimens varied among and within each CNS fungal infection
(6) two-year-survival was substantial for candidiasis and cryptococcosis but worse for aspergillosis and mucormycosis. CONCLUSIONS: Overall, patients with clinical suspicion of neuroinfection and neurologic deficits should be examined for fungal infections, especially if immunocompromised. Given their increasing frequency, CNS fungal infections will become more relevant in daily neurological practice.