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Oesophageal lymphangioma: an exceedingly rare tumour. An asymptomatic 57-year-old man was referred for evaluation of a 10 mm pale pink subepithelial polypoid lesion of the distal oesophagus; the lesion was found incidentally during an upper gastrointestinal endoscopy. The endoscopic ultrasound (EUS) revealed a heterogeneous, although primarily hypoechogenic, well-circumscribed lesion, located in the submucosa. Owing to the absence of endosonographic features, which would have allowed for a precise diagnosis, EUS-guided fine-needle aspiration was performed; however, the cytology was inconclusive because of the lack of cellular representation. We chose to remove it by cap-assisted endoscopic mucosal resection. However, due to its shape and deep location, complete removal was not achieved. A macrobiopsy was taken, and histological examination, of all oesophageal layers except the adventitia, revealed many irregularly dilated lymphatic vessels, beneath normal squamous epithelium. These findings are consistent with the diagnosis of lymphangioma. A follow-up EUS did not reveal any changes. The patient is currently asymptomatic and is under surveillance.