We describe a rare case of leiomyoma of the chest wall in a 55-year-old female. Computed tomography showed a well-circumscribed neoplasm with a diameter of 2 cm in the right chest wall. The tumor was excised with video-assisted thoracic surgery. Histopathology confirmed that the tumor was leiomyoma arising from the microvascular smooth muscle in the chest wall. We present the immunohistochemical profiles of the tumor in detail, critically reviewing the previously reported cases.