The authors report the association of a clinically florid form of lupus with malignant hypertension and renal failure. The renal histology revealed lesions of nephroangiosclerosis, with virtually no signs of lupus proliferative glomerulonephritis. With a follow-up period of 42 months, the renal function has returned to normal, although steroid treatment has been interrupted for 33 months. However, intensive antihypertensive treatment is still required. This unusual case is discussed in the light of previous reports in the literature of the association of disseminated lupus erythematosus and malignant hypertension, which is nevertheless a rare entity.