Scleroderma renal crisis is defined as the new onset of accelerated arterial hypertension and/or rapidly progressive oliguric renal failure. The pathogenesis is not well understood but there is increasing evidence that renin- angiotensin system is involved. We report an one female patient with diffuse scleroderma and renal crisis. Initial treatment with ACE inhibitor was not effective in controlling blood pressure until the temporal initiation of hemodialysis. Predialysis serum creatinine level was 8.4 mg/dL, but after initiation of hemodialysis, adequate control of blood pressure was achieved with ACE inhibitor alone. This case illustrated many features of the syndrome of scleroderma renal crisis and supported the early use of captopril and emergency hemodialysis if indicated.