Objective:To improve the differential diagnosis of systemic sclerosis (SSc) with hypertension and renal insufficiency.Methods:The clinical characteristics, diagnosis and treatment of a SSc patient with hypertension and renal insufficiency were reported and discussed.Results:A middle-aged female patient with a history of SSc for 5 years, headache and gross hematuria for 10 days was admitted. Abrupt increase in blood pressure and creatinine, glomerular hematuria, proteinuria, low complement C3 and C4, positive antinuclear antibody (ANA), anti-dsDNA antibody and perinuclear antineutrophil cytoplasmic antibody (pANCA) were presented. Renal pathology showed lupus nephritis (LN) (type Ⅳ). After glucocorticoid pulse therapy, followed by cyclophosphamide, belimumab, and symptomatic treatment, the symptoms were relievedand lupus disease activity were decreased.Conclusion:For SSc patients with increased blood pressure and creatinine, the presence of other diseases should be considered in addition to scleroderma renal crisis. Renal biopsy and pathological examination should be performed to confirm the diagnosis and avoid misdiagnosis.