Rationale: Pulmonary benign metastasizing leiomyoma (PBML) is rare, usually occurs in women who underwent hysterectomy during the reproductive years, and has no obvious clinical symptoms. A full understanding of the characteristics of PBML is important for its sequential treatment and prognosis. Patient concerns: In this report, a 36-year-old female patient with previous uterine leiomyoma who underwent 3 surgical resections of the uterus, bilateral fallopian tubes, and partial omentum was investigated. The physical examination revealed a tumor in the right lower lobe and mediastinum and a solid nodule in the right middle lobe. Diagnoses: Chest computed tomography (CT) confirmed a tumor in the right lower lobe and mediastinum and a solid nodule in the right middle lobe. Further positron-emission tomography computed tomography (PET-CT) with 18F-fluorodeoxyglucose (FDG) of the whole body showed mildly intense accumulation of 18F-FDG in the tumor (maximum standardized uptake value [SUV max], 2.6). A pathological examination then confirmed the presence of fibrous and vascular tissue after CT-guided percutaneous biopsy of the tumor in the right lower lobe. Additionally, surgical resection of the tumor and nodule was performed for histological analysis and immunohistochemical assays for estrogen receptor (ER) and progesterone receptor (PR). Interventions: The patient underwent complete tumor surgical resection and nodule wedge resection. Outcomes: No postoperative complications occurred. No recurrence or other signs of metastasis were found during an 18-month follow-up observation period. Conclusion: In this case, lung and mediastinal metastasis of uterine fibroids was observed. However, depending on only a postoperative histological analysis is insufficient for the diagnosis of PBML. Histological analysis combined with an evaluation of the expression levels of ER and PR is crucial for the diagnosis and treatment of PBML.