Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, encompassing granulomatosis with polyangiitis and microscopic polyangiitis, is a severe autoimmune inflammatory condition affecting various organs. These diseases are characterized by necrotizing vasculitis, with granulomatosis with polyangiitis also featuring granulomatous inflammation and multinucleated giant cells, unlike microscopic polyangiitis. The pathogenesis involves complex immune responses, including activation of the alternative complement pathway and neutrophil activation. Recent advancements in management have shifted from cytotoxic to targeted biological medications, improving disease remission and patient survival. Enhanced ANCA testing has facilitated earlier diagnosis, contributing to better outcomes. However, despite reduced acute mortality, long-term complications such as chronic kidney and cardiovascular diseases remain concerns. Current treatments still heavily depend on glucocorticoids, highlighting the ongoing need to develop less toxic therapeutic options. This summary underscores the evolution in understanding and managing ANCA-associated vasculitis, emphasizing the transition towards targeted therapies and the challenges of long-term morbidity and treatment toxicity.