SUMMARY Introduction. Localized central nervous system histoplasmosis is a rare clinical form of this systemic mycosis. It could be presented as chronic meningitis, focal encephalic or spinal cord lesions, stroke syndromes and encephalitis. Clinical case. We present a case of an immunocompetent child with localized histoplasmosis of the central nervous system. The neurological case had a chronic evolution and presented several focal neurological deficits. Magnetic resonance imaging showed multiple masses of the brainstem and the cervical spinal cord. Immunological tests showed antibodies anti-Histoplasma capsulatum. The clinical condition of the patient improved with the sequential treatment with amphotericin B and fluconazole. Conclusions. In spite of the rare presentation, central nervous system histoplasmosis should be considered in the differential diagnosis of multiple brain and spinal cord lesions in a patient resident in an endemic area. Immunological tests are useful as a diagnostic tool and the antifungal treatment could lead to cure.