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ANCA-associated vasculitides (AAVs) are small-vessel vasculitides with a broad spectrum of manifestations, since almost all organs can be affected. Miscellaneous organ manifestations including the involvement of cardiovascular and musculoskeletal system, gastrointestinal and urogenital tract, occur in only a minority of patients but may have a profound impact on morbidity and mortality. As an example, cardiac involvement, including subclinical forms, is emerging as a diffuse and insidious disease component. In eosinophilic granulomatosis with polyangiitis (EGPA), cardiac abnormalities have been related to eosinophil infiltration and occur in up to 50% of patients, accounting for the first disease-related cause of death. Overall, AAV patients experience an increased frequency of ischemic heart disease, due to “accelerated atherosclerosis.” Gastrointestinal manifestations are not specific and may range from mild to life-threatening complications, even requiring surgery. A small number of granulomatosis with polyangiitis (GPA) male patients develop prostatitis as the most common urogenital manifestation, while therapy based on cyclophosphamide may cause hemorrhagic cystitis and urothelial cancer. In isolated cases, muscle vessels may be affected by vasculitis resulting in chronic ischemia and fiber degeneration. Although the list of manifestations is potentially infinite, it must be kept in mind that AAV may associate with a number of autoimmune and fibro-inflammatory diseases. Thus, patients with AAV should be carefully and periodically evaluated for new or modified manifestations and should receive a serological screening to exclude the development of overlap syndromes.