We present a 7-year-old boy with chronic meningitis caused by Blastomyces dermatitidis. A review of the literature revealed 32 cases of central nervous system blastomycosis in children between 1983 and 2016, of which 18 represented parenchymal disease of the brain or spinal cord. Blastomycosis affecting the central nervous system is rare but should be considered in children with chronic meningitis.