Wegener's granulomatosis (WG) is frequently diagnosed based on respiratory and renal manifestations. Breast involvement is rare. To date, approximately 27 cases of breast involvement by WG have been published. Most cases present in women between the third and seventh decades are unilateral and are accompanied by systemic manifestations of the disease. Although breast tissue is rarely affected by WG, the clinical presentation of these lesions requires that breast carcinoma be excluded from the clinical differential diagnosis. This publication is the most extensive review of the entity in the English literature in the last 3 decades.