Exserohilum rostratum is a dematiaceous fungus that rarely causes infection in humans. We describe a patient with severe aplastic anemia who developed fatal disseminated disease caused by E. rostratum, and we review the English-language literature on this unusual agent. Our patient initially presented with signs and symptoms of sinusitis, which progressed to invasive pulmonary disease. The clinical course and histopathologic findings at autopsy were similar to those seen in cases of invasive aspergillosis or mucormycosis in that a propensity for vascular invasion and dissemination was seen.