Uterine leiomyomas have been reported to metastasize to various organs including the lungs, skeletal muscles, bone marrow, peritoneum, and heart. They may present with symptoms related to the metastases several years after hysterectomy. These tumors regress after menopause, and it is rare to detect active tumors in postmenopausal women. Despite their ability to metastasize, they are considered to be benign due to the lack of anaplasia. Pulmonary benign metastasizing leiomyoma is usually detected in the form of pulmonary nodules incidentally on imaging. Tissue biopsy of these nodules is required to identify them as benign metastasizing leiomyomas. Immunohistochemical analysis and molecular profiling may further help detect any malignant transformation in it. Untreated pulmonary benign metastasizing leiomyoma may result in the formation of cystic structures, destruction of lung parenchyma, and hemothorax and may cause respiratory failure. Surgical resection and hormonal therapy help prevent progression of this disease and provide an avenue for a cure.