This study describes 10 cases of IgM nephropathy in whom the main morphological findings consisted of diffuse mesangial deposition of IgM and varying degrees of mesangial cell proliferation. In addition, focal segmental sclerosis was present in 1 patient and global sclerosis in another. An ill-defined electron-dense deposit was seen within the mesangial area in 1 case. Except for 1 patient, who had hematuria only, all suffered from nephrotic syndrome without deterioration of renal function. In view of the constant and characteristic finding of a diffuse mesangial IgM deposition, it is suggested that this form of nephropathy constitutes an entity separate from focal glomerulosclerosis or minimal change disease.