Aspergillus, a ubiquitous mold, may cause invasive and fatal disease in immunosuppressed patients. Myelopathy is an uncommon presentation of invasive aspergillosis. This report describes three children admitted to the hospital between 1988 and 1995 who developed myelopathy as the first evidence of invasive aspergillosis. All had advanced leukemia and were profoundly immunosuppressed because of chemotherapy and broad-spectrum antibiotics. Weakness and pain presented first; then, sensation to pain and temperature was lost 2 to 6 days later, followed by complete myelopathy. Multiple brain lesions were seen on magnetic resonance imaging in one patient. Despite antifungal therapy, aspergillosis proved fatal within 1 month of onset of myelopathy in all patients. Physicians caring for immunocompromised children should be aware of myelopathy as a presentation of invasive aspergillosis.