Hypertensive emergency is a common cause of emergency room (ER) visits. Scleroderma renal crisis (SRC) is one of the rare causes of hypertensive emergency. SRC is a life-threatening condition that presents with acute onset severe hypertension accompanied by retinopathy, encephalopathy, and rapidly worsening renal function. We present a case of hypertensive emergency and renal failure with positive anti-Scl 70 and RNA polymerase III which is characteristic of SRC. Despite appropriate supportive care and timely treatment with angiotensin-converting enzyme inhibitors, the patient progressed to end-stage kidney disease.