Cunninghamella, a zygomycete in the order Mucorales, is an extremely rare cause of human infection. Of the five reported cases of human disease caused by this fungus, none involved rhinocerebral infection. Here, the authors document what appears to be the first case of rhinocerebral mucormycosis caused by Cunninghamella bertholletiae in an elderly man who had diabetes with sideroblastic anemia and hemochromatosis. The disease was rapidly fatal. The mycology and classification of this organism are presented, and the previous case reports in the literature are reviewed.