Idiopathic granulomatous mastitis (IGM) is a chronic, non-cancerous inflammatory breast disease primarily affecting young parous women. Characterized by sudden onset of breast lumps, abscesses, sinus tracts, and ulcers, IGM has a variable incidence and prevalence, with higher rates observed in certain racial and ethnic groups. Despite being non-cancerous, IGM can be highly aggressive and impact patients' quality of life. The etiology remains unclear, but it is increasingly recognized as an autoimmune disease, supported by clinical and histopathological findings. Key features include erythema nodosum, arthritis, and coexistence with other autoimmune diseases. Seasonal fluctuations in disease onset and triggers such as pregnancy, hormonal disorders, trauma, smoking, and microorganisms are also discussed. Cellular dysregulation, cytokine imbalances, and human leukocyte antigen (HLA) variations are implicated in the immune pathogenesis of IGM. Treatment options include watch-and-wait, corticosteroids, immunomodulators, and emerging biological agents targeting B cells, kinases, and cytokine inhibitors. However, the role of autoantibodies like ANA in IGM remains unexplained. Further research is needed to elucidate the etiology, immunologic mechanisms, and improve treatment strategies for IGM.Idiopathic granulomatous mastitis (IGM) is a chronic, non-cancerous inflammatory breast disease primarily affecting young parous women. Characterized by sudden onset of breast lumps, abscesses, sinus tracts, and ulcers, IGM has a variable incidence and prevalence, with higher rates observed in certain racial and ethnic groups. Despite being non-cancerous, IGM can be highly aggressive and impact patients' quality of life. The etiology remains unclear, but it is increasingly recognized as an autoimmune disease, supported by clinical and histopathological findings. Key features include erythema nodosum, arthritis, and coexistence with other autoimmune diseases. Seasonal fluctuations in disease onset and triggers such as pregnancy, hormonal disorders, trauma, smoking, and microorganisms are also discussed. Cellular dysregulation, cytokine imbalances, and human leukocyte antigen (HLA) variations are implicated in the immune pathogenesis of IGM. Treatment options include watch-and-wait, corticosteroids, immunomodulators, and emerging biological agents targeting B cells, kinases, and cytokine inhibitors. However, the role of autoantibodies like ANA in IGM remains unexplained. Further research is needed to elucidate the etiology, immunologic mechanisms, and improve treatment strategies for IGM.