An extremely rare case of cerebral phaeohyphomycosis caused by Exophiala dermatitidis is reported. We described an otherwise healthy young child whose presentation was a progressive intracranial hypertension and paraplegia, accompanied by urine retention and constipation. His blood test showed eosinophilia with the proportion of eosinophilic cell up to 28%. A computed tomography and magnetic resonance imaging of the brain and the spinal cord revealed multiple lesions. A cerebral biopsy was performed and the pathological report was cerebral phaeohyphomycosis. Cultures of the tissue and cerebrospinal fluid grew the same fungus. Extraction of genomic DNA from cultures was performed, and the DNA sequence displayed 99% sequence homologies with E dermatitidis. The patient's response to therapy was poor, and the boy died 2 months later. Our experience suggests that phaeohyphomycosis should be included in the differential diagnosis in children with multiple intracranial lesions of unknown origin and eosinophilia.