The involvement of central nervous system in paracoccidiodomycosis has rarely been described, with an incidence rate varying from 9.99% to 27.27%. There are two basic forms of clinical presentation: meningeal and tumor-like (abscesses, granulomas, nodules, and cysts). The Paracoccidioides brasiliensis is preferentially described in cerebral hemispheres, cerebellum, medulla oblonga and meninges, and exceptionally in the spinal cord. The authors present a case of paracoccidioidomycosis which diagnosis was achieved by microscopic examination of material from oral lesions and specific serology. The patient presented clinical signs of spinal cord involvement confirmed by lesions found in magnetic resonance imaging. They emphasize the inedit therapeutic response to a new antifungal agent (fluconazole) used for the first time in this kind of clinical manifestation, and the excellent prognosis when diagnosis is promptly made.