Objective: To report a case of spinal intramedullary blastomycosis causing myelopathy. Clinical presentation: An otherwise healthy 13-year-old patient was diagnosed with respiratory North American blastomycosis. She subsequently received a five-month course of itraconazole with presumed resolution of the infection. The patient presented again at 14 years of age with a lumbar myelopathy. Magnetic resonance imaging revealed an intramedullary lesion of 1 cm diameter at the level of T12-L1. Intervention: A T12-L1 laminectomy was performed with a gross total resection of the lesion. Pathological examination and microbiological culture of the specimen was consistent with blastomycosis. Postoperatively, the patient was placed on a five week course of amphotericin B. The patient showed substantial improvement in neurological function. Conclusion: Blastomycosis can present as an isolated intramedullary lesion causing compromised function. It should be considered in the differential diagnosis of a patient with a myelopathy and previously recognized blastomycosis. The prognosis is good with surgical resection.