HISTORY AND CLINICAL FINDINGS: A 67-year old patient underwent a kidney biopsy because of newly diagnosed hypertension, haemolytic anemia with fragmentocytes and acute kidney failure requiring dialysis therapy. The biopsy showed thrombotic microangiopathy. Since last winter Raynaud's phenomenon and changes of hands and lips were recognised. INVESTIGATIONS: Initial immunological tests revealed anti-nuclear antibodies (ANA) but neither anti-centromere nor anti-Scl70 antibodies. The positive analysis of anti-RNA polymerase III antibodies confirmed the clinical suspicion of scleroderma renal crisis in the setting of first diagnosis of systemic sclerosis. TREATMENT AND COURSE: After diagnosis therapy with lisinopril, candesartan and amlodipin was established. Four months after discharge dialysis dependency persisted. CONCLUSION: Scleroderma renal crisis is an important differential diagnosis in the setting of acute kidney failure. Medical history, clinical examination and immunological test confirm the diagnosis. The mainstay of therapy is aggressive blood pressure control with ACE-inhibitors (or angiotensin receptor blocking agents).