Background: Anti-neutrophil cytoplasm antibodies-associated vasculitis (AAV) is a rare autoimmune condition with high-relapsing rate and incidence of complications, resulting in increased morbidity and mortality. Characters of patients with anti-neutrophil cytoplasm antibodies-associated vasculitis in Saudi Arabia require further exploration. Objective: To evaluate the clinical profile, relapse rate and disease-related complications among patients with AAV at a tertiary hospital in Saudi Arabia. To estimate the role of BVAS score at the time of presentation in predicting relapse during the disease course. Design and Setting: This retrospective cohort study was performed through data collection from patients' records who had AAV, who visited the rheumatology clinic. The collected data involved the demographics of patients and their investigations, medications, and outcomes of treatment. Statistical analysis was executed through SPSS version 26. Results: Fifty-two patients were eligible for inclusion, while 48 patients were analyzed because of missing data. Females represented 60.4%. Half of the patients were more than 50 years old, and 68.8% had comorbidities. As for diagnosis, 62.5% had granulomatosis with polyangiitis, 25% had eosinophilic granulomatosis with polyangiitis, and 12.5% had microscopic polyangiitis. The rate of relapse was 31.3%, while the remission rate was 68.8%. Additionally, 66.7% had lower respiratory involvement, and 43.8% had renal involvement. More than half of the patients had BVAS score below 14.5 points. The study did not explore a positive correlation between the disease relapse and high BVAS at the first presentation. Conclusion: Early prediction of relapse and such intervention is of paramount importance in order to avoid accrual of organ damage with treatments that prevent further relapses. BVAS score was not found to be a potential predictor in our study. Future studies are highly endorsed, with prospective design and large sample size to achieve statistical significance for the incidence of relapses and complications.