Granular cell tumour of the oesophagus is rare and usually single. It is diagnosed by endoscopic appearance, results of endosonography, and histological examination of biopsy specimens. Although histological examination is required for diagnosis, it is difficult occasionally to obtain tumour samples by forceps because granular cell tumour is usually located in the submucosal layer. We report the case of a Japanese man with two granular cell tumours of the oesophagus. One lesion was diagnosed as a granular cell tumour by histological examination of a biopsy specimen, but the other was not. Endoscopic resection was performed to obtain the diagnosis and treat the lesions since some granular cell tumours are potentially malignant. Both tumours were completely resected endoscopically, and the diagnosis of granular cell tumour could be established by histological examination of resected tissue. Endoscopic resection is thus useful in the diagnosis and treatment of granular cell tumour of the oesophagus.