Pauci-immune necrotizing glomerulonephritis is a form of a rapidly progressive glomerulonephritis that is associated with the anti-neutrophil cytoplasmic antibodies (ANCA). ANCA-associated pauci-immune glomerulonephritis may be secondary to rheumatic diseases. We report on a patient with limited cutaneous systemic sclerosis who presented with subacute onset of nephritic syndrome and renal insufficiency. Her myeloperioxidase ANCA titer was elevated and renal biopsy confirmed pauci-immune crescentic glomerulonephritis, which was stabilized with high-dose glucocorticoid and cyclophosphamide. ANCA-associated necrotizing glomerulonephritis is rarely reported in systemic sclerosis and a systematic review is presented.