Of 140 patients with "scleroderma renal crisis" encountered during a 33-year period, 15 of 131 (11%) whose blood pressures were recorded were normotensive during this complication. In comparison with 116 patients with hypertension, the normotensive patients significantly more often had microangiopathic hemolytic anemia (90% versus 38%) and thrombocytopenia (83% versus 21%). Pulmonary hemorrhage occurred in 6 normotensive patients. More normotensive patients had received high doses of corticosteroids (prednisone greater than or equal to 30 mg/day) during the 2 months immediately preceding renal crisis (64% versus 16%). A role for corticosteroids in precipitating renal crisis is suggested. The 12-month survival was significantly reduced in the normotensive patients (13% versus 35%).