The incidence of fungal infections of the central nervous system (CNS) has gradually increased in recent years. Intracranial fungal infection can be classified as diffuse and focal infections. The clinical manifestations of these infections include fever and cranial pressure caused by meningitis or meningoencephalitis, as well as focal neurological defects caused by lesions in the intracranial space. Diagnosing fungal infections of the CNS requires comprehensive analysis of the patient’s medical history, epidemiology, underlying disease, clinical manifestation, imaging manifestations, and results from various laboratory tests. The identification of fungal bodies or structures in brain tissue or cerebrospinal fluid specimens is the golden standard of diagnosis. The principles for the treatment of fungal infections of the CNS are the effective control of pathogenic risk factors, use of effective antifungal drugs, and the active implementation of surgical intervention for fungal abscesses and granulomas. In the meantime, new diagnoses and treatments should be actively explored to improve the prognosis of patients.