Objective To study the clinical and pathological features of primary leiomyosarcoma of the breast. Methods One case of primary leiomyosarcoma of the breast were reported with clinical data, pathological features, phenotype of IHC,and review of literature. Results The primary leiomyosarcoma of the breast showed single mass, and didn't have any features of symptoms and auxiliary examination, and it was difficult to make correct diagnosis before operation. Histologic examination showed that the tumor was composed of relatively uniform spindly cells arranged in interlacing fascicles. The tumor cells proliferated with obvious atypia, and it had many mitotic rate. With IHC staining, the cells were positive forVimentin, Desmin and SMA. Conclusions Primary leiomyosarcoma of the breast is rare,but can be diagnosed properly based on pathological features. Surgical resection is the effective method.