Idiopathic granulomatous mastitis (IGM) is a rare form of chronic inflammatory breast disease. Although it is a benign breast lesion, it may be sometimes difficult to distinguish from breast cancer. The cause of IGM is unknown, but may be associated with autoimmunity, abnormal hormone levels and infection. While the clinical manifestations of IGM involve various manifestations of inflammation, the diagnosis is principally established by histopathology, characterized by non-caseating granulomas and microabscess formation centered on the breast lobules. Therapeutic options for IGM range from observation to various medical treatments, such as steroids, immunosuppressants, and antibiotics, to surgical intervention, particularly if secondarily infected. Given that the controversy on etiology and treatment choices, we accomplished the present review through reviewing IGM-related literature published in ‘Pubmed’ and ‘Web of science’ databases during 1997 to 2020, aiming to provide the basis for rational clinical diagnosis and treatment.