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Nephrotic syndrome without kidney injury revealing intravascular large B cell lymphoma. We describe the case of a 64-year-old woman admitted for fever of unknown origin, who developed nephrotic syndrome during hospitalisation and pulmonary infiltrates. Renal biopsy disclosed intracapillary glomerular invasion by intravascular large B cell lymphoma. Clinical and biological evolution was favourable after rituximab, cyclophosphamide, doxorubicine, vincristine and prednisone (R-CHOP) treatment and autologous stem cell transplant. Two years after diagnosis the patient was considered in remission.