Histoplasmosis is a disease caused by Histoplasma capsulatum var. capsulatum, which is endemic in Latin America. The manifestation of the disease in the central nervous system (CNS) is more frequent in immunosuppressed individuals with disseminated presentation. Pulmonary manifestations are usually the first symptoms. However, when neurological manifestations are the first clinical manifestations, the diagnosis becomes a challenge. The early diagnosis is fundamental in the final outcome of the patient. The objective is to report a case of difficult identification of cerebral histoplasmosis in a nonimmunosuppressed patient in Joinville, Brazil.