Tumours of the thoracic wall constitute a wide histological spectrum. However, the literature includes very few reports of a leiomyoma occurring at the extrapleural chest wall. In this report we present our experience together with a review of the literature. Our patient was a 33-year-old woman who was diagnosed with leiomyoma of the chest wall and treated accordingly and successfully. To the best of our knowledge, this is the seventh report in the literature defining the clinical entity.