Vasculitis of the breast (VB) may be an isolated finding or a manifestation of systemic vasculitis. In the current study we sought to characterize isolated VB (IVB) and compare it to VB in the setting of systemic vasculitis. We studied VB cases in the literature and patients cared for at our institution. We analyzed clinical, laboratory, and histologic features (including vessel size and type of inflammatory infiltrates); course of illness; biopsy procedure; and treatment. Based on the presence of localized or systemic disease at the time of disease presentation and during the follow-up, we divided patients into 3 groups: IVB (Group 1), VB with proven or indirect evidence of systemic vasculitis (Group 2), and VB with possible systemic involvement (Group 3). We identified a total of 34 cases of VB (30 from PubMed [National Library of Medicine, Bethesda, MD] and 4 from our pathology database). All patients presented with breast lesions, which were the only expression of disease in 16 (47%). Eighteen, 6, and 10 patients belonged to Group 1, 2, and 3, respectively. Constitutional symptoms were present less often in Group 1. Musculoskeletal symptoms occurred only in Groups 2 and 3. Patients in Groups 2 and 3 had higher erythrocyte sedimentation rates and lower hemoglobin levels, and also received corticosteroids more frequently than those in Group 1. No differences were found in the other analyzed parameters between groups. In summary, VB is uncommon, and in about half of the cases, occurs in the form of IVB. Histologic characteristics do not correlate with disease extent. In IVB patients, constitutional and musculoskeletal manifestations are usually absent. Such patients generally do not require systemic therapy and may be cured by resection alone.