A case of the nail-patella syndrome with nephropathy is presented. Despite moderately severe proteinuria and severe pathologic changes demonstrable by electron microscopy, renal function was normal. It is suggested that the initial lesion may be localized in the basement membrane, and that with progression of the disease glomerular proliferation leads to obstruction of the capillary lumen and hyalinization. A striking feature was the presence of electron-lucent areas within the thickened medial portion of the basement membrane, the significance of which is unknown. No prognostic evaluation regarding renal function can be drawn from this case or from the few available reported renal studies.