Idiopathic granulomatous mastitis (IGM) is a chronic benign inflammatory disease of the breast that may mimic breast cancer. It is most common in parous young fertile women, although it can occur in nulliparous women and in men. IGM is an idiopathic disease due to the influence of some environmental factors in genetically predisposed subjects. Several pathogenic hypothesis have been proposed in the last years (autoimmune, hormonal, infective genesis). IGM presents as a painful palpable mass located in one of the two udders. The skin is usually normal but could present signs of inflammation with or without lymph nodes involvement. Ultrasonography, mammography, magnetic resonance can be diagnosed an IGM, but pathog-nomonic radiological signs has not yet reported in literature. Biopsy findings show granulomatous lesion centered on the breast lobule, as in granulomatous mastitis induced by tuberculosis or sarcoidosis. The aim of this review of literature is to verify the development of new advanced diagnostic techniques and multidisciplinary approach for this condition. In the last years innovative approaches have modified IGM diagnosis and therapy, avoiding surgery in most of cases, introducing a more conservative medical approach based on recent etiopathological hypothesis. (www.actabiomedica.it).