Pulmonary benign metastasizing leiomyoma (PBML) represents a rare disease which is mostly diagnosed in reproductive-age women with a hysteromyomectomy history. This disease could occur in any age group, but more common in women of late fertility. And its clinical processes are closely linked to sex hormone levels. Here we describe a case of PBML simultaneously diagnosed with uterine leiomyoma at patient's first visit without a hysteromyomectomy history. The patient was a 37-year-old woman presenting with recurrent lower abdominal discomfort for more than half a year. No nausea, cough, dyspnea, hemoptysis, or abdominal distension was found. Additionally, the menstruation of the patient was regular. Image examinations confirmed a single solid pulmonary nodule in the right upper lobe, multiple fibroids of the uterus, and left adnexal nodules at her first visit. Laparoscopic hysteromyomectomy and pulmonary segmentectomy were sequentially performed with an interval of one month. Pathological findings were consistent with benign metastasizing leiomyomas. Estrogen and progesterone receptors were positive by immunohistostaining of the tumors. In conclusion, when confronted with female patients, who are at childbearing age with or without a history of uterine fibroids surgery, with single or multiple lesions in the lung that highly resemble metastatic tumors, but fail to find the primary malignant tumor clinically, the possibility of PBML must be considered. And sequential surgical treatment could be arranged.