ANCA-associated vasculitides (AAV) are rare diseases characterized by small-vessel necrotizing vasculitis, multiorgan involvement and positivity for antineutrophil cytoplasmic antibodies (ANCA). The main phenotypes are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), representing distinct yet partially overlapping entities in terms of pathogenesis, clinical expression and therapeutic management. Patients with AAV face a substantial cardiovascular (CV) and thrombotic risk, with higher rates of myocardial infarction, ischaemic stroke and venous thromboembolism than the general population. The excess CV burden reflects a complex, time-dependent interplay between disease-related inflammation, traditional cardiovascular risk factors and treatment-related toxicity, with inflammatory activity emerging as a key driver of early CV events. Across the disease course, this evolving risk profile requires multidisciplinary management to limit CV damage accrual and related mortality. This review integrates evidence on pathogenesis, clinical manifestations and management of CV disease in AAV, highlighting its time-dependent trajectory and key unmet needs.