A 58 year old man presented with skin manifestations of scleroderma without systemic involvement. Within few weeks of oral corticosteroids and penicillamine therapy, rapidly progressive systemic sclerosis developed. The deterioration manifested by skin lesions, diffuse interstitial restrictive lung disease, acute renal failure with normal blood pressure values, and microangiopathic hemolytic anemia compatible with hemolytic uremic syndrome. Chronic renal failure developed and was treated by dialysis, but the patient died due to sepsis. The course of renal involvement was normotensive; antihypertensive therapy was not prescribed even once. The association of scleroderma renal crisis with normal blood pressure is probably a rare and ominous combination.