ObjectiveTo re-understand the nerve sheath tumors of the breast, to improve its diagnosis and cure rate.MethodSearched the relevant literatures of nerve sheath tumors of the breast, to analyze and summarize the origin, etiology, clinical manifestations, imaging characteristics, pathological characteristics, treatment, and prognosis of this disease.ResultsNerve sheath tumors of the breast was a very rare disease, which originated in the neuromembrane Snowwang cells. its specific cause was unknown and clinical manifestations were not specific, and other breast diseases were difficult to identify, such as leaf ybrilloma, mammary vascular epidermal cytoma, breast fibroids, and so on. Imaging data could provide some reference value, but the gold standard relied on pathology and immunohistochemical examination. Surgery could cure benign nerve sheath tumors of the breast, but there was a possibility of malignant changes that required follow-up after surgery. Malignant neuroblastoma was mainly surgically removed, supplemented by chemotherapy, which could effectively prevent the recurrence of tumor and distant metastasis. The prevention of nerve sheath tumors of the breast could be referred to breast cancer screening.ConclusionsDuring clinical practice, we need to understand the diagnosis and treatment of nerve sheath tumors of the breast to achieve early detection, early diagnosis, and early treatment, as well as improve the diagnosis rate and cure rate of the disease, in order to protect women’s physical and mental health.