OBJECTIFS The association between paraneoplasic nephrotic syndrome and Hodgkin's disease is rare estimated to be about 0.5 to 1% (0.4% as minimal change disease and 0.1% as amyloid). MéTHODES: We report the case of an Hodgkin's disease revealed by an isolated nephrotic syndrome. RéSULTATS: A 34-year-old man presented with the clinical symptoms of nephrotic syndrome, Physical examination was notable for lower extremity edema. Though his hematologic evaluation was consistent with anemia (white blood cells = 11300/mm(3); hemoglobin, 7,9g/dL; platelets, 482000/mm(3)), there was no evidence of hemolysis but a biologic inflammatory syndrome. Nephrotic range proteinuria (3,4g in 24 hours), acute renal failure (creatinine 160umol/L), and hypoalbuminemia (23g/L) were also present. Kidney biopsy revealed endocapillary proliferative glomerulone-phritis associated with vasculitis. A treatment with prednisone was started with favorable response. Two months after the diagnostic of nephrotic syndrome was made, the patient presented prolonged fever and weight loss. The physical examination showed cervical lymphadenopathy, which lymph node biopsy revealed Hodgkin lymphoma of the nodular sclerosing type. The patient was treated for Hodgkin disease stage IVB with ABVD regimen, and was in complete remission of both Hodgkin's disease and nephrotic syndrome. CONCLUSION Causes of nephrotic syndrome in Hodgkin's disease can include renal vein thrombosis, amyloidosis or paraneoplastic syndrome. This case attracts attention that a rare cause of nephrotic syndrome can be Hodgkin's disease.