The authors describe a 51-year-old Japanese woman who developed nephrotic syndrome in association with a total hydatidiform mole. The nephrotic syndrome remitted completely following hysterectomy. A renal biopsy performed before the operation showed diffuse mesangial cell proliferation of a moderate degree, and thickened capillary walls with focal and segmental subendothelial deposits, as well as circumferential mesangial interposition. Occasional foci of the mesangiolysis were also observed. Immunofluorescence microscopy revealed granular staining of IgM along the glomerular capillary walls in a fringe pattern. A review of the literature revealed that this patient appears to be the first case of nephrotic syndrome associated with a total mole, although there have been two cases of nephrotic syndrome due to preeclamptic nephropathy associated with a partial or transitional mole.