Purpose: The controlling nutritional status (CONUT) score was developed to detect undernutrition in patients. Here, we investigated whether the CONUT score estimated at diagnosis could help predict poor outcomes [all-cause mortality, relapse, and end-stage renal disease (ESRD)] of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Materials and Methods: We retrospectively reviewed and collated data, including baseline characteristics, clinical manifestations (to calculate AAV-specific indices), and laboratory results, from 196 newly diagnosed AAV patients. Serum albumin, peripheral lymphocyte, and total cholesterol levels (at diagnosis) were used to calculate CONUT scores. Results: In total, 111 patients had high CONUT scores (>= 3), which showed higher frequency of myeloperoxidase-ANCA and ANCA positivity, and demonstrated higher AlV-specific indices. The optimal cut-offs of CONUT score (at diagnosis) for predicting all-cause mortality and ESRD were >= 3.5 and >= 2.5, respectively. Patients with CONUT scores higher than the cut-off at diagnosis ex- hibited lower cumulative and ESRD-free survival rates compared to those with lower scores than the cut-off. In multivariable analyses, diabetes mellitus [hazard ratio (HR): 4.394], five-factor score (HR: 3.051), and CONUT score >= 3.5 (HR: 4.307) at diagnosis were independent predictors of all-cause mortality, while only serum creatinine (HR: 1.714) was an independent predictor of ESRD occurrence. Conclusion: CONUT score at diagnosis is associated with all-cause mortality in AAV patients.