Paracoccidioidomycosis of the central nervous system presents either as meningeal or pseudotumoral lesions. Although occurring more frequently in the brain and meninges, they can occasionally involve the spinal cord. A case of paracoccidioidomycosis in the cervical spinal cord is reported in this paper. Difficulties in establishing the etiologic diagnosis, the importance of radiologic examination of the thorax, and the treatment of the patient are commented. In an extensive review of the literature on the subject, only three other cases have been found, which are also discussed.