Rapidly progressive glomerulonephritis corresponds to a clinical scenario of glomerular damage involving rapid deterioration of renal function with multiple etiologies. Within these, 85 % of cases are associated with pauci-immune vasculitis with antineutrophil cytoplasmic antibodies (ANCA). However, a remaining subgroup may present ANCA-negative results. The clinical presentation often involves a decline in the glomerular filtration rate, sometimes requiring renal replacement therapy, as well as extrarenal manifestations such as diffuse alveolar hemorrhage. Confirmatory diagnosis is achieved through renal biopsy, negative serology for ANCA, and exclusion of other etiologies. According to international clinical practice guidelines, the recommended treatment for both entities is the same, with the addition of renal replacement therapy if necessary. However, the prognosis tends to be unfavorable with little to no recovery of renal function. This case presents a female patient in the early decades of life with ANCA-negative, rapidly progressive, pauci-immune glomerulonephritis.

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