We describe the medical management of isolated renal zygomycosis in an adult patient with AIDS during chemotherapy for AIDS-related lymphoma. After initial presentation during the first cycle of chemotherapy, the infection was contained within the kidney following recovery of the neutrophil count without medical or surgical intervention. Since he was not considered to be a candidate for nephrectomy, his infection was treated with amphotericin B lipid complex during subsequent chemotherapy. Neutropenia was minimized by the addition of cytokine support therapy with granulocyte colony-stimulating factor and reduced doses of chemotherapy. Following this strategy, his lymphoma completely resolved, and renal zygomycosis was controlled. At the time of this writing, he had been in complete remission for 18 months without evidence of progressive fungal infection. This report and our literature review indicate that isolated renal zygomycosis can be associated with a favorable prognosis, occurs with greatest frequency in patients with AIDS, is associated with parenteral access, and may be managed by medical therapy alone.