Objective To explore the incidence, diagnosis and treatment of nephrotic syndrome ( NS ) following allogeneic hematopoietic stem cell transplantation ( allo-HSCT) in one single center.Methods Analysis were carried out based on the records of 1464 patients following allo-HSCT hospitalized in Peking University People's Hospital from January 1996 to April 2009, so as to evaluate the incidence and outcomes of NS. A total of 1464 patients whose survival time was longer than 100 d were enrolled. Eight patients (0.6%) had NS following allo-HSCT. Using the nested case-controlled study, 56 cases who received transplantation within the same period were selected as control group for risk factors analysis. Results The median time of NS was 488(54-1185)d after allo-HSCT. Lower extremities' edema and heavy proteinuria were present in all the patients. Two patients were accompanied with impaired renal function. Six patients suffered from chronic graft-versus-host disease (GVHD). Three patients with NS were accompanied with other symptoms of GVHD. Renal biopsy examination showed typical features of minimal change diseases in 3 patients and membranous nephropathy in 1 patient. All cases achieved complete remission (CR) after taking combined immunosuppressant agents. One patient relapsed soon after all the immunosuppressant agents were stopped. Six patients were still surviving with disease free. One patient died of the relapse of leukemia and another patient died of early discharge from hospital after allo-HSCT. The possible factors correlated with the occurrence of NS were analyzed, and the occurrence of chronic GVHD was identified as an independent factor of NS. Conclusions NS should be carefully taken into consideration when edema and proteinuria are present in patients following allo-HSCT. The occurrence of NS is probably related with chronic GVHD. NS responds well to immunosuppressant agents.