The authors report on a case of fibro-leiomyoma of the upper oesophagus. The patient presented dysphagia and oppressive retrosternal pain. Computed tomography and magnetic resonance of the thorax revealed a solid voluminous formation at the level of the posterior upper mediastinum. The US-endoscopy showed that this was a tumour originating from the esophageal wall with macroscopic characteristics of benignity, suggestive of a leiomyoma. The patient was treated by thoracotomy enucleation of the large tumour after sectioning the azygous vein on the same side as the lesion. Histological examination of the surgical resection confirmed that the tumour was a fibro-leiomyoma of the esophagus with a conspicuous vascular component and an interstitial inflammatory focus. The patient now appears to be asymptomatic and illness-free.