Purpose: Invasive mycosis caused by the Aspergillus, Fusarium, and Mucor can be fetal, especially in the immunocompromised patients with central nervous system (CNS) involvement. Here we present a case of CNS Fusarium infection, and this is the first reported case of Fusarium brain abscess in Taiwan., Case Report: A 65-year-old woman presented with fever and conscious disturbance for 3 days. Neurological examination showed stupor consciousness, neck stiffness, multiple cranial nerves palsy, and bilateral Babinski signs. Magnetic resonance imaging showed multifocal lesions involving medulla oblongata, pons, bilateral cerebral peduncles, and bilateral cerebellar peduncles. Cerebrospinal fluid (CSF) study revealed neutrophil predominant pleocytosis, but both blood and CSF culture were negative. We treated patient with ceftriaxone and vancomycin initially as empiric therapy for suspected bacterial meningoencephalitis. However, chronic sinusitis with fungal ball and brain abscess were later found. Despite antifungal treatment and surgical intervention, patient expired 3 months after admission. Fungal culture of the brain abscess disclosed Fusarium species 2 weeks after her death., Conclusion: CNS Fusarium infection should be considered when an immunocompromised patient presenting with fever, conscious change, cranial nerve palsies, and angioinvasion suggested by brain imaging. To properly manage the disease, early effective antifungal therapy and neurosurgical intervention are important.